Somerset, Wiltshire, Avon and Gloucestershire (SWAG) Cancer Services. Skin Cancer Network Site Specific Group. Clinical Guidelines.

Somerset, Wiltshire, Avon and Gloucestershire (SWAG) Cancer Services Skin Cancer Network Site Specific Group June 2017 Revision due: April 2019 Page 1 of 70

VERSION CONTROL THIS IS A CONTROLLED DOCUMENT. PLEASE DESTROY ALL PREVIOUS VERSIONS ON RECEIPT OF A NEW VERSION. Please check the SWCN website for the latest version available here. VERSION DATE ISSUED SUMMARY OF CHANGE OWNER S NAME 0.1 19 th April 2015 First draft SWAG Skin SSG 0.2 6 th June 2015 Formatting Helen Dunderdale 0.3 26 th June 2015 Updated referral and Amrit Darvay imaging guidelines 0.3 29 th June 2015 Addition to introduction Anita Takwale referring to previous Three Counties Guidelines plus minor amendments 1.0 30 th June 2015 Finalised SWAG Skin SSG 1.1 5 th May 2016 Amendment to section Amrit Darvay 2.3.13 follow up 1.2 April 2017 Biennial review and addition of Appendix 1: Guidelines for the follow up of immunocompetent adult patients with skin cancer, 2016 SWAG Skin SSG The staging guidelines for malignant melanoma will be updated in the near future Page 2 of 70

This document was edited by: Amrit Darvay, Chair of the SWAG Skin SSG, Consultant Dermatologist, North Bristol NHS Trust Helen Dunderdale, SWAG Cancer Network SSG Support Manager Thanks to Drs Adam Bray, Katherine Finucane, Amarnarth Challapalli, Joe Boyle, Rachel Wachmuth, Graham Collin, Leigh Biddlestone and Mr Dominic Ayres and Mr Antonio Orlando for reviewing and rewriting some of the guidelines. These clinical guidelines have been agreed by: Name Position Trust Date agreed Dr David de Berker Consultant Dermatologist University Hospitals Bristol NHS Foundation Trust (UH June 2017 Dr Bill Phillips Dr Rachel Wachsmuth Dr Jill Adams Dr Tom Millard Mr Antonio Orlando Consultant Dermatologist Consultant Dermatologist Consultant Dermatologist Consultant Dermatologist Consultant Plastic Surgeon Bristol) Royal United Hospitals Bath NHS Foundation Trust (RUH) Yeovil District Hospital NHS Foundation Trust Taunton and Somerset NHS Foundation Trust Gloucestershire Royal Hospitals NHS Foundation Trust North Bristol NHS Trust June 2017 June 2017 June 2017 June 2017 June 2017 Page 3 of 70

Skin NSSG Contents Contents Measures Page Section 1 Introduction 10 The NSSG agreed Clinical A08/SC/LS-16-004 2 Guidelines for the 11 Treatment of Skin Cancer 2.1 Clinical Management of Basal Cell Carcinoma 11 2.1.1 Referral Guidelines and Process 13 2.1.2 Cellular Pathology 14 2.1.3 Multidisciplinary Teams (LS/SSMDT) 2.1.4 Data 15 2.1.5 Definitive Treatment of Primary or Recurrent Lesions 15 2.1.6 Adjunctive and Complicated Disease Management 15 2.1.7 Staging Investigations 16 2.1.8 Children and Young People 16 2.1.9 Oncology Provision Children and Young People 16 2.1.10 Supportive and Palliative Care 16 2.1.11 Follow up 17 2.1.12 Mohs BCC flow chart and Guidelines 18 2.2 Management of Primary Cutaneous Squamous Cell Carcinoma (SSC) 19 2.2.1 Pathway Review 19 2.2.2 Overall Objectives 20 2.2.3 Scope 21 2.2.4 Referral Guidelines and Process 21 2.2.5 Clinical features 22 2.2.6 Pathological features 22 2.2.7 Imaging features 25 15 Page 4 of 70

2.2.8 Sentinel Lymph Node Biopsy 25 2.2.9 Referral to Multidisciplinary Team 25 2.2.10 Therapeutic Interventions 26 2.2.10.1 Surgery 26 2.2.10.2 Chemotherapy 27 2.2.10.3 Radiotherapy 28 2.2.10.4 Reduction of immunosuppression 2.2.10.5 Systemic Retinoids 28 2.2.11 Follow-up 28 2.2.12 LS/SSMDT 29 2.2.13 Data 29 2.2.14 Specialist MDTs 31 2.2.15 Oncology Provision Children and Young People 31 2.2.16 Guidance for Follow up of Patients Treated for 33 Squamous Cell Skin Cancer 2.3 Clinical Management of Malignant Melanoma 34 2.3.1 Malignant Melanoma Pathway Overview 34 2.3.2 Process and Terminology 35 2.3.3 Referral Guidelines and Process 2.3.4 Local/Specialist MDT 36 2.3.5 Cellular Pathology 36 2.3.6 Multi-disciplinary Meeting (MDM) 36 2.3.7 Data 37 2.3.8 2.3.9 Definitive Treatment of a Primary Lesion Specialist Skin Cancer MDT (SSMDT) 2.3.10 Children and Young People 39 2.3.11 Oncology Provision 39 2.3.12 Supportive and Palliative Care 39 2.3.13 Follow Up 40 28 35 37 38 Page 5 of 70

3 Imaging in Skin Cancer 40 3.1 Malignant Melanoma 40 3.2 Squamous Cell Carcinoma and Basal Cell Carcinoma 41 3.3 Merkel Cell Tumours 41 3.4 Enquiries 41 4 Histopathology 42 4.1 Introduction 42 4.2 The multi-disciplinary team meetings 4.3 Specimen Types 43 4.4 Turnaround time in reporting 43 4.5 Specimen examination 44 4.6 4.7 Grading and staging of skin tumours Use of ancillary laboratory techniques 4.8 Audit 46 4.9 Referral for review or specialist opinion 4.10 Additional staff costs 46 4.11 TNM 7 46 4.12 Minimum dataset for reporting 47 4.13 Enquiries 47 5 Skin Cancer Surveillance and Management of Immuno- Suppressed Organ Transplant 5.1 Introduction 47 5.2 5.3 5.4 5.5 Referral Pathway For Solid Organ Transplant Patients and Immunosuppressed Patients With Suspected Skin Cancer Patients With Confirmed Skin Cancer Patients With Multiple/Rare Skin Cancers 5.6 Surveillance 49 5.7 Policy 49 42 45 45 46 47 47 48 48 48 Page 6 of 70

5.8 Compliance 49 5.9 Treatment 49 5.10 Policy 49 5.11 Compliance 50 5.12 Guidelines 50 6 Oncology in Skin Cancer 50 6.1 6.2 6.3 6.4 6.5 6.6 6.7 6.8 6.9 6.10 SCC and BCC Treated With Primary Radiotherapy Joint Protocol for the Treatment of Skin Cancers with Radiotherapy Indications for adjuvant Radiotherapy Radiotherapy For Primary/Locally Recurrent BCC and SCC Procedure for Post-Operative or Primary Radical Radiotherapy Procedure for Post-Operative Nodal Melanoma Procedure for Post-Operative Nodal Melanoma Radiotherapy Palliative Radiotherapy for Melanoma Radiotherapy for Lentigo Maligna Radiotherapy for Cutaneous Lymphoma 6.11 Merkel Cell Tumour 56 6.12 Kaposi s Sarcoma 56 6.13 Cutaneous Anglosarcoma 56 6.14 Keloids 57 6.15 Skin Metastasis 57 6.16 7 Options for Type of Radiotherapy Clinical Management of Rare Skin Tumours Including Skin Lymphoma 7.1 Introduction 57 50 51 52 52 53 54 54 55 55 55 57 57 Page 7 of 70

7.2 Dermatofibromasarcoma Protuberans 7.2.1 Clinical Behaviour 58 7.2.2 Diagnosis and Investigation 58 7.2.3 Level of Care 58 7.2.4 Treatment 58 7.2.5 Radiotherapy 59 7.2.6 Chemotherapy 59 7.2.7 Follow-up 59 7.3 Atypical Fibroxanthoma 59 7.3.1 Clinical 59 7.3.2 Pathology 60 7.3.3 Treatment 60 7.3.4 Follow-up 60 7.4 Kaposi s Sarcoma 61 7.4.1 Introduction 61 7.4.2 Classic Kaposi s Sarcoma 61 7.4.3 Endemic Kaposi s Sarcoma 61 7.4.4 Transplant Related Kaposi s Sarcoma 61 7.4.5 Epidemic Kaposi s Sarcoma 61 7.4.6 Level of Care 61 7.4.7 Clinical Features 61 7.4.8 Diagnosis 61 7.4.9 Staging 62 7.4.10 T (tumour) status 62 7.4.11 Treatment 63 7.5 Merkel Cell Carcinoma Key Points 63 7.5.1 Introduction 63 7.5.2 Level of Care 65 7.5.3 Management 65 7.5.4 Investigation 65 7.5.5 Metastatic Disease 66 7.5.6 Treatment 67 7.5.7 Follow-up 68 58 Page 8 of 70

7.6 Clinical Management of Cutaneous Lymphoma 7.6.1 Local Skin Cancer MDT 68 7.6.2 Referral Pathways 69 7.6.3 Management 69 7.6.4 Haemato-oncology Links 70 7.6.5 Referral Pathway 70 7.6.6 Supranetwork MDT for Total Skin Electron Beam Therapy 7.6.7 Photophoresis 70 7.7 Clinical Management of Palpable Lymph Nodes in Someone with a Previous 71 Diagnosis of Skin Cancer 7.7.1 Protocol for Assessment of Palpable Lymph Nodes in Someone with a Previous 71 Diagnosis of Skin Cancer 7.7.2 The NSSG guide to Management of Palpable 72 node Flow Chart 8 Appendix 1: Guidelines for the follow up of immunocompetent adult patients with skin cancer, 2016 72 68 70 1. Introduction The following guidelines pertain to the local management of skin malignancies for the Somerset, Wiltshire, Avon and Gloucestershire (SWAG) Network Skin Cancer Site Specific Group (NSSG). They have been edited from the previous ASWCS network guidelines. Gloucestershire Hospitals previously contributed and referred to the Three Counties Network Guidelines. These have yet to be scrutinised to establish whether there are any differences in the management of skin cancer across the two networks. A process of communicating any changes that require incorporating will take place over the coming year. The NSSG refers to the most recent National Institute for Health and Care Excellence (NICE) Skin Cancer clinical guidelines and will amend guidance if necessary on publication of the new melanoma guidelines later in 2015. http://www.nice.org.uk/guidance Page 9 of 70

Primary care clinicians should refer to the NICE guidelines Suspected Cancer: recognition and management of suspected cancer in children, young people and adults (2015) for the signs and symptoms relevant when referring to skin cancer services. Further details on the two week wait referral process can be found in the NSSG constitution. The NSSG is committed to offering all eligible patients entry into clinical trials where available. Consent to provide tissue for research purposes will also be sought wherever appropriate. Page 10 of 70

2. The NSSG agreed for the Treatment of Skin Cancer (A08/SC/LS-16-004) 2.1 Clinical Management of Basal Cell Carcinoma Process and Terminology Scope Page 11 of 70

This document sets out the pathway of care (PoC) for patients with suspected or proven Basal Cell Carcinoma. It has been updated to ensure compliance with the 2006 and updated 2010 NICE Improving Outcomes Guidance for People with Skin Tumours including Melanoma (IOG). The IOG states that networks provide three levels of care for patients with skin cancer. These are: Primary Care Local hospital skin cancer Multidisciplinary Team (LSMDT) Specialist skin cancer MDT (SSMDT). Based on the population of Bristol, Gloucestershire, Somerset and Wiltshire, the SWAG NSSGs proposes that there are three skin cancer SSMDTs in the region, based at North Bristol NHS Trust (Brunel Building, Southmead Hospital), Taunton and Somerset NHS Trust (Musgrove Park Hospital) and Gloucestershire Hospitals NHS Foundation Trust that function at both Local and Specialist Level (LS/SSMDT) in addition to local hospital MDTs (LSMDT) at each Acute Trust working to the protocols and policies agreed by the network. This document describes the process/care available at each level and is compliant with the 2006 IOG. Tumours The scope of this pathway of care is confined to: High Risk Basal Cell Carcinoma (HRBCC) Definition: Primary Cutaneous Basal Cell Carcinoma (BCC) is a common malignancy arising in the basal cells of the epidermis and its appendages. It is locally invasive and only very rarely has the potential to metastasise. Clinical subtypes: Nodulo-ulcerative Superficial Morphoeic Fibroepithelioma Pigmented. Low risk BCCs may be managed by practitioners in Primary Care who are appropriately trained, accredited and are members of the LS/SSMDT or in Secondary Care. Low risk tumours are those not on the head and neck and are smaller than 2cm. Management of high risk tumours should take place in Secondary Care. High risk tumours are those on the head and neck and all tumours of 2cm in diameter or larger. Page 12 of 70

2.1.1 Referral Guidelines and Process BCC usually presents as a slowly developing indurated area or nodular tumour that may ulcerate. The lesions can occasionally only be confidently differentiated from other nonmelanoma and melanoma skin cancers on histology. General practitioners are encouraged to refer patients with BCCs to secondary care for assessment and management. Where there is a primary care skin cancer service commissioned by the CCG this may also be an option for low risk BBCs. Consider routine referral for people if they have a skin lesion that raises the suspicion of a basal cell carcinoma Only consider a suspected cancer pathway referral (for an appointment within 2 weeks) for people with a skin lesion that raises the suspicion of a basal cell carcinoma if there is particular concern that a delay may have a significant impact, because of factors such as lesion site or size. Follow the NICE guidance on improving outcomes for people with skin tumours including melanoma: the management of low-risk basal cell carcinomas in the community (2010 update) for advice on who should excise suspected basal cell carcinomas. [new 2015] The BCC sub-group recommend that lesions identified as HRBCC should not be managed in Primary Care. Lesions not thought to be BCC in origin and biopsied in Primary Care that prove to be a high risk BCC should be referred to Secondary Care. A copy of the histology report should be attached to the referral letter. If a photograph of the pre-biopsy lesion was taken, a copy could accompany the referral if possible. High Risk BCCs considered at high risk have a greater recurrence (and/or rarely metastasis) after treatment. Based on clinical features apparent to the GP at assessment: Sites (listed in decreasing metastatic potential): Other factors: Head and neck. Size > 20 mm Previously treated and recurrent disease Immunosuppressed patients Page 13 of 70

Genetic disorders (e.g. Gorlins/Basal Cell Nevus syndrome). Based on histological features apparent after incision or excision biopsy: Histologic subtype: Morphoeic Infiltrative Micronodular Basosquamous. Histologic features: Perineural invasion Invasion below dermis. Secondary Care Patients should be seen by a member of the dermatology team. Patients will: Undergo a thorough loco regional skin examination Have the lesion photographed if indicated Have a biopsy taken or be booked for biopsy/removal as indicated. Be considered for Mohs Micrographic Surgery if high risk Have an excision biopsy - with margin according to national guidelines. Incisional/punch/curette or shave biopsy of the lesion may also be performed as appropriate. The network has accepted the guidelines for management published by the British Association of Dermatologists. Telfer NR, Colver GB, Morton CA. Br J Dermatol 2008; 159: 35-48 The patient will receive information on the further management plan when histology is available. 2.1.2 Cellular Pathology It is expected that pathology departments will adhere to guidelines on specimen handling described in the Royal College of Pathology Minimum Data Set for Skin Cancer (RCP MDS). Patients with BCCs are not currently included in the Department of Health (DoH) targets. Histology departments are not obliged to report on these lesions within 2 weeks of biopsy unless doubt exists in the clinical diagnosis. The NSSG proposes a 2 calendar week turnaround time for all skin malignancy to reflect the need for prompt patient feedback and also to manage instances where a different diagnosis such as squamous cell carcinoma is revealed. Page 14 of 70

The NSSG will monitor adherence to both the guidelines set out in the RCP MDS and targets through the process of regular audit. 2.1.3 Multidisciplinary Teams (LS/SSMDT) Selected patients with high risk BCCs should be discussed at multi-disciplinary team (MDT) meetings. These meetings will normally be held at least two weekly. Patients may be discussed by more than one MDT as they progress along their pathway of care. 2.1.4 Data All decisions on patients with BCC discussed by the MDT will be recorded by the team. The MDT will be responsible for validating data, especially data required for cancer registration purposes. Completeness and quality of data should be reviewed by MDTs at their compulsory annual operational policy meeting. 2.1.5 Definitive Treatment of Primary or Recurrent Lesions Treatment should proceed according to national guidelines. Treatment modalities may include: Excision with appropriate clinical margin Topical agents (imiquimod/fluorouracil) Cryotherapy/cryosurgery (in special circumstances) Photodynamic therapy (PDT) Radiotherapy Curettage and cautery Mohs micrographic surgery (see under Adjuvant Treatment) Systemic therapies (rarely) Palliative and supportive care. 2.1.6 Adjunctive and Complicated Disease Management: Adjuvant Treatment Mohs micrographic therapy is appropriate for certain high risk tumours. This is a complex technique available through the Dermatology & Plastic Surgery Department at Southmead Hospital, Bristol for the SWAG area. Some patients within the region may still prefer to travel to other centres for geographical reasons (e.g. Exeter, Oxford). The NSSG will only support other adjuvant therapies undertaken in the context of Specialist Skin Cancer MDTs. Oncologists will act as the gate-keepers for these therapies. Vismodegib is now licensed and available in the NHS for metastatic or unresectable BCC. Refer to the SSMDT for consideration. Metastatic and Nodal Disease (Rare) Page 15 of 70

Metastatic BCC is an extremely rare but devastating complication of this cancer with a poor survival outcome. Any patient with metastatic disease will be discussed with the MDT oncologists for further management via SSMDT. See above re. Vismodegib. Sentinel Node Biopsy (SNB) has no role in BCC management. 2.1.7 Staging Investigations Staging investigations are not normally required in BCC. Only when metastases are suspected should staging investigations be arranged. These are defined in the network guidelines for radiology. These may only be requested at the discretion of the SSMDT and when the patient has accepted treatment. MRI or CT can occasionally be useful when bulky intracranial or bony disease is suspected. 2.1.8 Children and Young People Children and Young People (CYP) with basal cell carcinoma will be treated in accordance with principles set out in the CYP IOG. All children and Young People up to the age of 16 must be referred to the CYP Principal Treatment Centre which for SWAG is the paediatric dermatology service at the Bristol Royal Hospital for Children. Gloucestershire Hospitals have a separate paediatric service. All Young adults between 16 and 24 years must be offered the choice of referral to the CYP Principal Treatment Centre, but may be seen within the Dermatology service framework which varies for different parts of the network but will normally be the normal dermatology clinic. Referral to a CYP Principal Treatment Centre does not necessarily mean that treatment will be undertaken at that centre; shared care management protocols may allow some treatments to be undertaken locally. 2.1.9 Oncology Provision Children and Young People The Non-Surgical Oncological management of all patients with high risk BCC will be recommended by the SSMDT or LSMDT where an oncologist is present. 2.1.10 Supportive and Palliative Care All patients with BCC will have access to appropriate specialist palliative care and support at every stage of the patient journey. Open and frank discussions with patients should take place with patients at all stages of their journey so that patients are not confused about their prognosis or have unrealistic expectations of any of the forms of treatment offered to them. Page 16 of 70

Relatives and carers will need to be supported and given appropriate information. However, in accordance with the recommendations set out in other Improving Outcomes Guidance, relatives and carers should not be given information different to that given to the patient. Palliative care provision should be available for all patients: Hospital teams, including the Clinical Nurse Specialists (CNSs) as for melanoma patients Primary Health Care Team would provide for palliative care at home General Practitioner should be informed within 24 hours of the diagnosis and treatment plan The management of symptoms, psychological, social and spiritual issues, the communication of the diagnosis, and any associated problems, should be within the domain of all health care professionals. Referral to specialist palliative care services should be considered when these issues have not been resolved and in particular for patients with: Complex symptom management issues Difficulties in adjusting to the diagnosis or disease progression Psychological and family issues such as communication problems within the family Spiritual issues such as the challenging of belief system/faith/cultural values as a result of the cancer. Consideration of specialist palliative care or support should be given throughout the patient pathway, particularly: At the Multidisciplinary Team Meeting When no active treatment is considered After active treatment At relapse In the terminal stages. 2.1.11 Follow up Long term hospital based follow-up of all patients after treatment of BCC is not necessary. Early detection and treatment improves outcomes of patients with recurrent or new primary disease. Studies suggest: 82% of local recurrences occur within five years 36% of patients develop new primary tumours 20% of high risk patients (skin type and exposure) develop multiple new lesions Long term follow-up may be appropriate in primary or secondary care or primarily led by patient self-examination The key purpose of follow up is patient education There is good evidence that well informed patients detect their own recurrence Page 17 of 70

Patients who do detect recurrence should be referred back to Secondary care Patients will have access to a clinical nurse specialist for support and advice Patients will have access to appropriate information and should always be given relevant discharge information Patients with in-situ/non-invasive and low risk lesions will be reviewed following appropriate treatment of the primary lesion for education and advice. Routine long term follow-up is not required All other BCCs should be reviewed and may be discharged at an appropriate time following patient education and agreement. The follow up period should be individualised Patients with Gorlin s syndrome or immunosuppression would usually be expected to require long-term follow-up. Long-term follow up should be undertaken at the discretion of clinicians and only after discussion and in agreement with patients. Follow up, where required, could be nurse led. When the MDT has agreed that adjuvant therapy is appropriate, follow up will be tailormade to suit the needs of the patient. 2.1.12 The NSSG refer to the Mohs for BBC flow chart and Mohs Guidelines. 2.2 Management of Primary Cutaneous Squamous Cell Carcinoma (SSC) Page 18 of 70

2.2.2 Overall Objectives This evidence based guideline for management of primary cutaneous SCC will: help practitioners to more reliably identify the high-risk tumours which are most likely to metastasise help to direct available resources to the management of patients with high-risk SCC, thus reducing the incidence of metastatic SCC The guideline recommendations will also help address the following concerns: Page 19 of 70

treatment variability amongst practitioners currently managing SCC that patients with high-risk SCC are not always referred to MDT meetings the limitations of the current TNM classification in identifying those SCC most likely to metastasise. This guideline provides recommendations for referral, management and follow up of patients aged 18 years and over with primary invasive SCC including SCC arising: on both sun-exposed (SE) and non-se sites in immunocompetent or immunosuppressed patients on historically accepted high-risk sites including the ear, lip (including the external mucosal lip) and scars in Bowen s disease in chronic wounds or areas of chronic inflammation. The guideline excludes: actinic keratoses keratoacanthoma as the Royal College of Pathologists have re-classified keratoacanthoma as well differentiated SCC of keratoacanthoma type. squamous intra-epidermal carcinoma/carcinoma-in-situ (Bowen s disease) Mucosal sites including internal mucosal lip recurrent SCC SCC arising in patients with cancer-predisposing genodermatoses such as xeroderma pigmentosum, recessive dystrophic epidermolysis bullosa, multiple self-healing squamous epithelioma of Ferguson Smith disease or albinism. 2.2.3 Scope This document sets out the pathway of care for patients with suspected or proven invasive Squamous Cell Carcinoma. These guidelines are extensively drawn from SIGN 140 Management of Primary Squamous Cell Carcinoma produced by the Scottish Intercollegiate Guidelines Network (www.sign.ac.uk), and from guidance from the British Association of Dermatologists. As new up to date national guidance is published the network will review its recommendations as necessary. The link to the document is here. Guidelines in bold type are either Page 20 of 70

recommendations on interventions that should be used and there is strong evidence that for the vast majority of patients the intervention(s) will do more good than harm or conditional recommendations on intervention(s) that should be considered and there is strong evidence that the intervention(s) will do more good than harm for most patients. The choice of intervention is therefore more likely to vary depending on a person s values and preferences, and so the healthcare professional should spend more time discussing the options with the patient. 2.2.4 Referral Guidelines and Process IDENTIFYING HIGH-RISK TUMOURS Consider a suspected cancer pathway referral (for an appointment within 2 weeks) for people with a skin lesion that raises the suspicion of squamous cell carcinoma as per NICE guidance June 2015. Invasive SCC usually presents as a rapidly developing indurated area or nodular tumour that may be keratinizing, crusted or ulcerative. KA frequently displays a classical clinical picture and history, but cannot always be confidently differentiated from other non-melanoma skin cancers, even on histology. All clinicians should endeavour to identify high-risk tumours at the earliest opportunity, and when referring patients with suspected SCC, should include details of the high-risk clinical features: immunosuppression, tumour diameter and site. General practitioners are encouraged to refer patients with a suspected SCC as a matter of urgency under the National Cancer 2-Week Wait rule (2-WW). The SCC sub-group recommends that lesions thought to be invasive squamous cell carcinoma should not be biopsied in Primary Care. Primary care clinicians should not knowingly biopsy a SCC, but if so, a copy of the histology report should either be faxed with the referral or accompany the patient to the clinic appointment. The case will be discussed at the multi-disciplinary meeting (MDM) in the first instance. 2.2.5 Clinical features SCCs considered at high risk have a greater recurrence and/or metastatic rate after treatment. Immunosuppression should be considered a high-risk clinical feature in patients with primary squamous cell carcinoma. Patients who are immunosuppressed are more likely to develop multiple primary SCCs and there is increased risk for an individual SCC to behave aggressively. Healthcare professionals Page 21 of 70

treating immunosuppressed patients need to be alert to the possibility of cutaneous malignancies and to exercise meticulous care at every stage of their SCC management. Immunosuppression, when present, should be indicated as part of the histopathology request form for skin biopsies from patients with suspected SCC. The ear should be considered the highest risk tumour site in patients with primary squamous cell carcinoma. Nose, cutaneous lip, eyelid and scalp tumour sites should be considered as high-risk features in primary squamous cell carcinoma. Squamous cell carcinoma arising within a site of skin trauma, e.g. burns, scar tissue, or a radiotherapy field, or within a site of pre-existing skin disease, e.g. venous leg ulceration or Bowen s disease, should be considered as high-risk SCC. The following features should prompt early referral: High levels of cumulative PUVA photochemotherapy Rapid tumour growth Field cancerisation Poorly defined clinical margins Pain/dysaesthesia. Clinically determined horizontal tumour diameter of >20 mm should be considered a highrisk feature in patients with primary squamous cell carcinoma. 2.2.6 Pathological features Tumour depth (in mm) and anatomical level should be reported as components of the core minimum dataset for primary squamous cell carcinoma. The tumour depth should be measured in the same way as Breslow depth is measured for melanomas, i.e. from epidermal granular layer or ulcer base to deepest contiguous tumour cell. For tumours with a papillomatous surface architecture, measurements should be taken from the bottom of epidermal troughs, rather than the tip of peaks, to avoid an overestimation of tumour depth. The measurement should be recorded to the nearest 0.1 mm. The use of the term Breslow depth should be avoided in pathology reports for SCC and reserved for melanoma. Pathology reports should state clearly whether the tumour is limited to the dermis or invades subcutaneous fat. If additional structures such as bone, skeletal muscle or cartilage are involved, this should be stated in the pathology report. The use of the term Clark level should be avoided in pathology reports for SCC. Tumour depth >4 mm should be considered a high-risk feature in patients with primary squamous cell carcinoma with depth >6 mm indicating a very high-risk tumour. Page 22 of 70

Tumour extension beyond the dermis into or through subcutaneous fat should be considered a high-risk feature in patients with primary squamous cell carcinoma. Tumour horizontal diameter of >20 mm should be considered a high-risk feature in patients with primary squamous cell carcinoma. The maximum diameter (to the nearest mm) of the macroscopic specimen should be reported as an essential component of the core minimum dataset for primary squamous cell carcinoma. Perineural invasion should be considered a high-risk feature in patients with primary squamous cell carcinoma. Presence or absence of perineural invasion should be reported as a component of the core minimum dataset for primary squamous cell carcinoma. Reporting of extent of perineural invasion and the size of the largest nerve branch involved is desirable. Lymphovascular invasion should be considered a high-risk feature in patients with primary squamous cell carcinoma. Presence or absence of lymphovascular invasion should be reported as a component of the core minimum dataset for primary squamous cell carcinoma. Non-sun-exposed sites (e.g. perineum), sites of previous irradiation, thermal injury, chronic ulcers, inflammation, sinuses, or Bowen s disease are all at higher risk of developing SCC. Desmoplastic subtype should be considered a high-risk feature in patients with primary squamous cell carcinoma. To categorise an SCC as being of desmoplastic subtype, at least one third of the tumour should show the desmoplastic phenotype, i.e. strands and nests of tumour cells surrounded by a prominent fibrous stromal response. Tumour subtype should be reported as part of the core minimum dataset for primary squamous cell carcinoma. Consideration should be given to treating the following tumour subtypes as high-risk variants of primary squamous cell carcinoma: Adenosquamous Spindle cell carcinoma Pseudoangiosarcomatous Acantholytic. Differentiation status should be reported as part of the core minimum dataset for primary squamous cell carcinoma. In line with the Royal College of Pathologists dataset, a three-item system should be used when reporting tumour differentiation in primary squamous cell carcinoma: Page 23 of 70

Well differentiated Moderately differentiated Poorly differentiated. A combination of the following morphological features should be used in the assessment of differentiation: Degree of keratinisation Presence/absence of intercellular bridges Degree of nuclear pleomorphism Number and nature of mitoses. By convention, tumour grade is assessed on the most poorly differentiated area in the tumour. Poorly-differentiated tumour status should be considered a high-risk feature in patients with primary squamous cell carcinoma. Where the tumour is present at the margin (the margin is involved) the case should be referred for discussion at the skin cancer MDT. Where the tumour margin is close (<1 mm) to deep or peripheral excision margins and there are other high-risk features present, cases should be discussed at the skin cancer MDT for consideration of re-excision or radiotherapy. The recommendation from the MDT will vary according to the site, size and number of highrisk features present. For many high-risk SCCs, an apparent pathological clearance margin of 1 mm would be considered insufficient. For some very high-risk SCCs, the recommendation will be for a clinical margin of 6-10 mm. Where the apparent clearance margin is close (<1 mm) to deep or peripheral excision margins in low-risk tumours further excision may not be required. These cases should usually be discussed at the relevant MDT. The nearest peripheral and deep excisional margin should be measured to the nearest 0.1 mm and should be reported as a component of the core minimum dataset for primary squamous cell carcinoma. For orientated excisions, it is desirable to comment on which peripheral margin(s) is/are involved, or are closest to the tumour edge. 2.2.7 Imaging features Imaging to determine the extent of a primary tumour may be appropriate in selected patients as determined by the MDT. This would include patients who have symptoms suggestive of perineural invasion or clinical evidence of bony erosion or at sites considered to be very high risk, for example arising on or around the ear. Where undertaken, regional lymph nodes may also be imaged. Page 24 of 70

2.2.8 Sentinel Lymph Node Biopsy For patients with SCC, sentinel lymph node biopsy should be conducted as part of a clinical trial only. 2.2.9 Referral to Multidisciplinary Team All patients with high risk SCCs should be discussed at the MDM. These meetings will normally be held at least every two weeks. Patients may be discussed at more than one MDM as they progress along their pathway of care. Where any of the following high-risk features are present, patients with primary SCC should be discussed at a skin cancer multidisciplinary team meeting: SCC arising on the ear Tumour diameter >20 mm Tumour thickness >4 mm Tumour extension beyond dermis into or through subcutaneous fat Perineural invasion Poorly differentiated Desmoplastic subtype Immunosuppression Recurrent SCC Established or suspected metastatic SCC Nose, external lip, eyelid and scalp tumour site Association with special clinical situations i.e. non-sun-exposed sites (e.g. perineum), sites of previous irradiation, thermal injury, chronic ulcers, inflammation, sinuses or Bowen s disease Adenosquamous histological subtype Spindle cell histological subtype Pseudoangiosarcomatous histological subtype Acantholytic histological subtype Lymphovascular invasion Tumour excision margins involved at deep or peripheral margins. MDT discussion is desirable where: Page 25 of 70

A tumour is at a surgically challenging site the referring clinician requests discussion due to specific clinical management issues, such as an SCC arising in patients with cancer-predisposing genodermatoses e.g. xeroderma pigmentosum, recessive dystrophic epidermolysis bullosa, multiple selfhealing squamous epithelioma of Ferguson Smith disease or albinism or where there is cognitive impairment or significant medical comorbidities. All SCC including low risk SCC should be reported on a minimum dataset which ensures that all high-risk SCCs are fast tracked to the MDT. Data on all SCC should be subject to clinical audit and sent to the Cancer Registry. 2.2.10 Therapeutic Interventions 2.2.10.1 Surgical Techniques Treatment choices should be discussed with patients taking account of the risks and benefits in functional and aesthetic outcomes balanced against clinical outcomes. The aim of surgery for squamous cell carcinoma should be complete histological clearance at peripheral and deep margins. To achieve adequate deep clearance, the surgeon should excise at the anatomical plane deep to the clinically apparent level of tumour invasion. This anatomical plane will vary according to tumour site. For high-risk tumours a clinical peripheral margin of 6 mm or greater is indicated, where surgically achievable and clinically appropriate. For low-risk tumours a clinical peripheral margin of 4 mm or greater is indicated where surgically achievable and clinically appropriate. An adequate diagnostic biopsy (incisional ellipse or wedge) can be helpful for planning the most appropriate treatment. When clinical clearance is uncertain, a delayed reconstruction pending the results of paraffin wax histology may be prudent. Mohs micrographic surgery should be considered at the multidisciplinary team meeting, for selected patients with high-risk tumours where tissue preservation or margin control is challenging, and on an individual case basis for patients with any tumour at a critical anatomical site. Use of conventional frozen section histology in high-risk SCC is not advised. Destructive Techniques Double / triple curettage and cautery can be considered for patients with low-risk SCCs, if healthcare professionals have had appropriate training with a blunt curette. Curettage and cautery is not suitable for high-risk SCC and should not be used where there are any high-risk clinical features. If the dermis is breached during curettage then the procedure should be converted to formal excision. Page 26 of 70

If the pathology report indicates any high-risk feature the patient should be referred to a skin cancer MDT for consideration of further treatment, since histological margins cannot be assessed. Photodynamic therapy should not be used for treatment of primary squamous cell carcinoma. 2.2.10.2 Chemotherapy Systemic chemotherapy for the management of patients with primary cutaneous SCC should not be used outside of a clinical trial. Systemic chemotherapy may be appropriate for patients with metastatic SCC. Discussion at SSMDT will be needed 2.2.10.3 Radiotherapy Primary radiotherapy should be considered for individual patients where surgical excision would be extremely challenging or difficult to perform or would be likely to result in an unacceptable functional or aesthetic outcome. Radiotherapy should be delivered by a clinical oncologist with a special interest in the management of skin cancer including SCC. Adjuvant radiotherapy should be considered for patients with a high risk of local recurrence or with close or involved margins where further surgery may be associated with increased risk of complications including functional or aesthetic morbidity. 2.2.10.4 Reduction of immunosuppression In organ transplant recipients with high-risk SCC, particularly those with multiple tumours or recurrent disease, minimisation or substitution of immunosuppression should be considered at a skin cancer MDT and discussed with the patient s transplant physician where appropriate. 2.2.10.5 Systemic Retinoids Selected patients who have developed multiple SCCs following renal transplantation should be considered for low-dose acitretin treatment (10-30 mg/day) for secondary prevention. Healthcare professionals should be aware that adverse effects are common, are dose related and may lead to dose reduction or discontinuation of treatment 2.2.11 Follow Up (see Table 1) Patients with SCC with any high-risk features should be offered follow-up appointments every four months for 24 months following treatment. One or two further appointments at three years may be appropriate depending on the clinical risk. Very high risk tumour follow up, as decided by the clinician with overall responsibility for the patient, could be extended Page 27 of 70

to a 5 year follow up with primary care involvement. There needs to be cooperation between primary and secondary care regarding patient follow up as not all patients can be reviewed by members of the LSMDTs and SSMDTs. Patients treated for low-risk SCC should be offered a review appointment to check histopathology (if not previously assessed), conduct skin surveillance and facilitate patient education in self-examination and skin cancer prevention, if not previously undertaken. Patients who are immunosuppressed and those who are developing multiple SCC should be offered long-term follow up. Advice on sensible photo-protection measures and self-skin examination should be offered to all patients. Ongoing follow up may be undertaken by an appropriately trained general practitioner with a specialist interest in dermatology or by a clinical nurse specialist. This is an opportunity to detect further primary skin cancers. The psychological impact of skin cancer should be considered at follow up and patients referred for psychological support as appropriate. 2.2.12 LS/SSMDT It is discretionary to discuss all patients at the MDT. It is required to discuss all those for whom the tumour is recurrent, incompletely excised or high risk in some other respect. The MDT will comply with NICE Guidelines with regard to composition and function. These will normally be held at least two weekly. Patients may be discussed at more than one MDM as they progress along their pathway of care. The Skin Cancer MDT co-ordinator role will be consistent with the job descriptions for other disease site MDT co-ordinators within the SWAG Network. The MDT co-ordinator will ensure that: All new cases of high risk invasive squamous cell carcinoma are discussed at MDT MDT decisions are communicated to GP within 24 hours of the MDT Data items described by the urgent skin cancer clinic proforma and RCP skin cancer proforma are accurately recorded MDT information is forwarded to the South West Cancer Registry A documented initial or revised plan of care will be made at MDMs. 2.2.13 Data Page 28 of 70

All decisions on patients with SCC discussed by the MDT will be recorded by the team. The MDT will be responsible for validating data, especially data required for cancer registration purposes. The MDT responsible for a particular element/episode of care along the patient pathway of care is the MDT responsible for the collection and validation of the data items associated with those elements/episodes. It is the responsibility of individual MDT lead clinicians and MDT co-ordinators to ensure that robust mechanisms for data collection and validation are in place. All decisions on patients with skin cancer discussed by the MDT will be recorded by the team. The MDT will be responsible for validating data, especially data required for cancer registration purposes. It is the responsibility of the team looking after the patient at a specific stage on the pathway of care to collect validated data for that patient at that specific stage of the journey. Data items for collection: Cancer Outcomes and Dataset for SCC NHS Number ICD-10 disease site code SnoMed histology code Staging (see Annex number 4) Co-morbidity: Dementia/Cerebrovascular disease Cardiovascular disease COPD/Asthma Liver failure/cirrhosis Diabetes Mental illness Other malignancy. First definitive treatment modality: Radiotherapy Brachytherapy Page 29 of 70

Chemotherapy Surgery (for skin cancer this includes diagnostic/therapeutic excision - if complete) Active surveillance No active treatment Specialist Palliative Care. 30 day post-operative death: Cause And if available: Date of death Cancer related Treatment related Process Other. Wherever possible data should be collected electronically Completeness and quality of data should be reviewed at least annually (but preferably six monthly) by MDTs at their compulsory annual operational policy meeting. 2.2.14 Specialist Skin Cancer MDTs: Children and Young People See NICE Quality Standard 55 published February 2014 which covers the provision of all aspects of cancer services for children and young people with cancer. For this quality standard, children are defined as aged 0 15 years and young people as 16 24 years. Children and Young People (CYP) with Cutaneous SCC will be treated in accordance with principles set out in the CYP IOG. All children and young people up to the age of 18 must be referred to the CYP Principal Treatment Centre which for the SWAG network is based at the Bristol Royal Hospital for Children. Gloucestershire have a separate paediatric service. Young People between 16 and 24 years will usually be seen in the normal Dermatology Clinic. Referral to a CYP Principal Treatment Centre does not necessarily mean that treatment will be undertaken at that centre; shared care management protocols may allow some treatments to be undertaken locally. Page 30 of 70

2.2.15 Oncology Provision Children and Young People The non-surgical oncological management of all patients with high risk SCC will be recommended by the SSMDT or the LSMDT where an Oncologist should be present. Supportive and Palliative Care All patients with SCC will have access to appropriate specialist palliative care and support at every stage of the patient journey. Open and frank discussions with patients should take place with patients at all stages of their journey so that they are not confused about their prognosis or have unrealistic expectations of any of the forms of treatment offered. Relatives and carers will need to be supported and given appropriate information. However, in accordance with the recommendations set out in other Improving Outcomes Guidance documents, relatives and carers should not be given information different to that given to the patient. Frail and terminally ill patients with SCC should always be discussed with the specialist palliative care team. Palliative care provision should be available for all patients: Hospital teams, including the Clinical Nurse Specialists as for melanoma patients Primary Health Care Team would provide for palliative care at home General Practitioner should be informed within 24 hours of the diagnosis and treatment plan. The management of symptoms, psychological, social and spiritual issues, the communication of the diagnosis, and any associated problems, should be within the domain of all health care professionals. Referral to specialist palliative care services should be considered when these issues have not been resolved and in particular for patients with: Complex symptom management issues Difficulties in adjusting to the diagnosis or disease progression Psychological and family issues such as communication problems within the family Spiritual issues such as the challenging of belief system/faith/cultural values as a result of the cancer. Consideration of specialist palliative care or support should be given throughout the patient pathway, particularly: At the Multidisciplinary Team Meeting When no active treatment is considered Page 31 of 70

After active treatment At relapse In the terminal stages. 2.2.16 Guidance for Follow up of Patients Treated for Squamous Cell Skin Cancer (Table 1 ) Site of tumour Histology and character Years of follow-up In secondary care In primary care Clinic frequency per year of follow up Faces, limbs and sun exposed sites Plus Well differentiated* 0 1 Year 1: 4 monthly Plus 1. Poorly differentiated 0 2 Year 1: 4 monthly 2. >2 cm Year 2: 6 monthly 3. Scar site Year 3: 6 monthly 4. Previous Rx 5. >4mm depth or perineural involvement 1 2 & 3 Lip and Ear Plus Well differentiated* 1 2&3 Lip and Ear 1. Poorly differentiated 1 & 2 3,4 & 5 Year 1: 4 monthly Year 2: 6 monthly Year 3: 6 monthly Year 1; 4 monthly 2. >2cm Year 2: 6 monthly 3. Scar site Year 3: 6 monthly 4. Previous Rx Year 4: 6 monthly Year 5: 6 monthly 5. >4mm depth or perineural involvement 1 & 2 3.4 & 5 Organ transplant recipients should be followed for recurrence and new skin tumours during the period of their immunosuppression. Clinician to be agreed with transplant physician. 2.3 Clinical Management of Malignant Melanoma 2.3.1 Malignant Melanoma Pathway Overview Page 32 of 70

2.3.2 Process and Terminology: Scope Page 33 of 70

Note NICE will publish new guidance on the management of melanoma in the summer of 2015 and the NSSG will update local guidelines in the light of NICE recommendations. This document sets out the pathway of care for patients with suspected/proven malignant melanoma. It is compliant with the 2006 NICE Improving Outcomes Guidance for People with Skin Cancer including Melanoma (IOG) and the Manual of Cancer Services 2008 Tumours The scope of the pathway is confined to malignant melanoma. 2.3.3 Referral Guidelines and Process Referral guidelines are based on the new NICE guidance published in June 2015. Refer people using a suspected cancer pathway referral (for an appointment within 2 weeks) for melanoma if they have a suspicious pigmented skin lesion with a weighted 7 point checklist score of 3 or more. Weighted 7 point checklist Major features of the lesions (scoring 2 points each): Change in size irregular shape irregular colour. Minor features of the lesions (scoring 1 point each): largest diameter 7mm or more inflammation. Refer people using a suspected cancer pathway referral (for an appointment within 2 weeks) if dermoscopy suggests melanoma of the skin. Consider a suspected cancer pathway referral (for an appointment within 2 weeks) for melanoma in people with a pigmented or non pigmented skin lesion that suggests nodular melanoma. General practitioners are encouraged to refer patients where melanoma is a possible diagnosis as a matter of urgency under the national 2 week wait rules. It is recommended that lesions thought to be melanoma should not be biopsied/excised in primary care. Lesions biopsied in primary care, that turn out to be melanoma should be referred under the 2 week wait rules. A copy of the histology should be attached to the referral and if a pre Page 34 of 70

biopsy photograph was taken, this should also be attached. These patients will be discussed at the MDT. 2.3.4 Local/Specialist MDT Patients will be seen within two weeks in a skin cancer clinic or an appointment identified as a skin cancer appointment. Patients will be assessed including: Reassurance if clinically benign Photography of the lesion for recording where warranted Thorough examination including loco-regional lymph node status where indicated Excision biopsy if not undertaken already and if necessary. Diagnostic excision biopsy ensuring a 2-5mm margin of normal skin and a cuff of sub-dermal fat is recommended. Incisional biopsy may be appropriate in certain circumstances. The visit will be documented. The patient will receive appropriate discharge information. When a patient is given a diagnosis of melanoma the clinician will ensure that the GP is informed of the diagnosis by the end of the following working day. 2.3.5 Cellular Pathology It is expected that pathology departments will adhere to guidelines on specimen handling described in the Royal College of Pathology minimum data set for skin cancer (RCP MDS). In order to comply with the DoH 31 day (referral to diagnosis) target histology departments are obliged to report on pigmented lesions within two weeks of biopsies taken from patients referred urgently with possible skin cancer. The network pathology cancer support group and the network site specific group (NSSG) for skin cancer will jointly monitor adherence to both the guidelines set out in the RCP MDS and the 31 day targets through process of regular audit. 2.3.6 Multi-disciplinary Meeting (MDM) All patients with melanoma should be discussed at multi-disciplinary meetings (MDM). The MDM will comply with NICE guidelines and the Manual of Cancer Services 2008 with regard to composition and function. Patients may be discussed at more than one MDM as they progress along their pathway of care. A key worker is to be allocated to the patients and the name of this person will be recorded in the patient s notes. Page 35 of 70

The skin cancer MDT co-ordinator role will be consistent with the job descriptions of other disease site MDT co-ordinators within the Avon, Somerset and Wiltshire network and will comply with the Manual of Cancer services 2008. The MDT co-ordinator will ensure that: 2.3.7 Data All new cases of melanoma are discussed at an MDM Data items are recorded on the South West skin cancer registry accurately A documented or revised plan of care is made at the MDM. All decisions on patients with melanoma discussed at the MDM will be recorded. The MDT will be responsible for validating data, in particular data required for cancer registry purposes. The MDT responsible for a particular element/episode of care along the patient pathway of care is the MDT responsible for the collection and validation of the data items associated with those elements/episodes. It is the responsibility of individual MDT lead clinicians and MDT co-ordinators to ensure that robust mechanism for data collection and validation are in place. Whilst MDTs are at liberty to collect more data items than those indicated below, there is an expectation that they will collect all of the following items: NHS number ICD-10 disease code SnoMed histology code Staging First definitive treatment modality (surgery, radiotherapy, chemotherapy, palliative care) 30 day post-operative death (cause) Date of death if available (cancer related, treatment related, other). Completeness of the data should be reviewed at least annually (but preferable more frequently) at the MDT compulsory annual operational policy meeting and by the South West Cancer registry. 2.3.8 Definitive Treatment of a Primary Lesion Initial excision biopsy will be with a clinical margin of 2-5mm. Lentigo maligna and other in-situ melanomas have no potential for metastatic spread and therefore lesions with clear histological margins require no further treatment. Further reexcision of scar to achieve 5-10mm clearance may be proposed by the MDT. For all invasive melanomas: 10mm margin will be taken for <1.0mm Breslow thickness Page 36 of 70

20mm margin will be taken for >1.01mm Breslow thickness Greater than 20mm margin have no current proven benefit and are not recommended. Melanocytic lesions of uncertain malignant potential will be managed as potential melanomas. The NSSG will review this recommendation as new evidence becomes available. 2.3.9 Specialist Skin Cancer MDT (SSMDT): Adjuvant Therapy Adjuvant therapy will be undertaken with oncologists acting as the gate-keepers. This will be undertaken in the context of the specialist skin cancer MDT, or LSMDT where an oncologist is present. Patients eligible for current clinical trials of adjuvant therapy will be identified and entry into trials will be co-ordinated by the oncologists. This includes chemotherapy, signal transduction inhibitors (e.g. Vemurafenib) and immunotherapy (e.g. Ipilimumab). Details will be published in the NICE guidelines. Nodal Disease Patients with nodal disease should have fine needle aspiration (FNA) cytology before undergoing block dissection. Block dissection should only be undertaken in the context of the specialist skin cancer MDT. Staging Investigations See details in section Imaging in Skin Cancer Sentinel Lymph Node Biopsy Sentinel node biopsy (SNB) is undertaken as part of multicentre trials or through consideration of special factors at the SSMDT. The NSSG aims to maintain sufficient activity in order to retain expertise and provide the service where required. SWAG policy on SNB will be finalised with the publication of the NICE melanoma guidelines later in 2015. 2.3.10 Children and Young People Patients with melanoma who are under the age of 19 should have mandatory individual case review by the SSMDT. The NICE guidance, Improving Outcomes in Children and Young People with Cancer, provides clear direction regarding the diagnosis and treatment of patients up to 24 years old within the South West. All children <19years will be referred to the South West Principal Treatment Centre for Children or Teenagers and Young Adults (TYA) (Bristol). Those less than 16 will be managed by the paediatric oncologist in conjunction with the shared care centres and SSMDT. Those 16-18 will be referred to the Teenage and Young Adult MDaT (Multi Disciplinary Advisory Page 37 of 70

Team) as well as the SSMDT and their care will be in the principal treatment centre (Bristol) with shared care locally as appropriate. Young adults 19-24 will be referred to the TYA MDaT as well as the appropriate LSMDT/SSMDT and have a choice of treatment in the principal treatment centre (Bristol) with local shared care or locally alone. 2.3.11 Oncology Provision After discussion at the LS/SSMDT, patients may be referred to the appropriate oncologist for discussion of oncological treatment. 2.3.12 Supportive and Palliative Care All patients with melanoma will have access to appropriate specialist palliative care and support at every stage of the patient journey. Open and frank discussions should take place with patients at all stages of their journey so that patients are not confused about their prognosis or have any unrealistic expectations of any of the forms of treatment offered to them. Relatives and carers will need to be supported and given appropriate information. However, relatives and carers will not be given information that is different to that given to the patients. Frail and terminally ill patients may be discussed with the specialist palliative care team. The management of symptoms, psychological, social and spiritual issues, the communication of diagnosis, and any associated problems should be within the domain of all health professionals. Referral to specialist palliative care services should be considered when these issues have not been resolved. Consideration of specialist palliative care or support should be given at all stages of the patient journey especially: At the MDT discussion When no active treatment is considered After active treatment e.g. electrochemotherapy At time of relapse In the terminal stages of the disease. 2.3.13 Follow Up Patients will be followed up according to the recommendations in the Melanoma NICE Guidance 2015, which are summarised in an algorithm on page 28 here. 3. Imaging Guidelines for Skin Cancer Page 38 of 70

3.1 Malignant Melanoma Stage I No routine imaging is indicated. Stage II IIIA Consider sentinel node biopsy. Stage IIIB to IV Ultrasound for characterisation, localisation and FNA of nodes. Baseline imaging for staging: Post contrast CT head and body, including neck in patients with head and upper torso disease and upper thighs in leg and lower torso primary disease. MRI To define clinically suspected disease in an anatomical location to assist in surgical planning. PET-CT To assess for distant metastatic disease when radical surgery is contemplated Confirmation of suspected recurrence or metastatic disease on basis of equivocal lesion on CT/MRI that is not readily amenable to minimally invasive biopsy Confirmation that an established single metastasis on another modality is indeed single prior to planning mode of surgical resection Response to therapy when part of a trial or relevant clinical management. Follow-up Follow up Patients who have documented nodal spread to neck/axillae or groins or other high risk patients likely to relapse with non-palpable disease: Consider follow-up with CT head and body 6 monthly for 2 years then 12 monthly for 3 years. Any stage Consider cross sectional imaging (US/CT/MRI) to investigate specific signs or symptoms. Nodal or distant Recurrence Page 39 of 70

Ultrasound and FNA to initially characterise/ diagnose nodal metastatic disease. Followed by staging CT as for patients with stage IIIB and greater disease. 3.2 Squamous Cell Carcinoma and Basal Cell Carcinoma Imaging not routinely required In locally advanced disease evaluate extent of primary disease with MRI or CT For staging utilise CT of affected and adjacent body compartment to include locoregional nodes Consider PETCT with equivocal CT or MRI or for trials inclusion or exclusion criteria In the absence of clinically enlarged nodes, ultrasound guided FNA cytology may be useful in evaluating regional lymph nodes in patients with high risk tumours. 3.3 Merkel Cell Tumours Stage 1 US +/- FNA of relevant lymph node group. Consider Sentinel Node Biopsy and staging CT Stage 2 as for Stage IIIb Melanoma. 3.4 Enquiries All enquiries relating to this document should be addressed to: Graham Collin (graham.collin@nbt.nhs.uk) Consultant Radiologist Brunel Building, Southmead Hospital 4. Histopathology 4.1 Introduction The Pathologist plays a central role in the diagnosis and staging of skin cancers. The information in their reports can help in the planning of future treatment of the patient. The IOG states in respect to the SSMDT: "Ideally there should be at least two specialist dermatopathologists or histopathologists with a special interest in dermatopathology. This is to provide flexibility and adequate cover during leave periods. There should be a designated lead in the area and ideally a deputy lead. The lead and deputy lead engaged in reviewing and reporting SSMDT skin cancer cases should each attend over 50% of SSMDTs. Other histopathologists reviewing and reporting SSMDT work should be able to demonstrate some MDT activity. All specialist histopathologists reviewing and reporting common and rare skin cancers should be able to demonstrate experience, competency and skills sufficient to fulfil the task, or undertake appropriate training to acquire the skills. The level of competence and skills for this activity is broadly that of the RCPath Diploma in Dermatopathology and American Board Page 40 of 70

Certification in dermatopathology. These qualifications are not, however, regarded as mandatory. All specialist histopathologists engaged in this work should participate in some CPD relevant to common and rare skin cancers and participate in an appropriate EQA scheme. Ideally this should be a national specialist EQA scheme in dermatopathology, when available. Those reporting primary cutaneous lymphoma must participate in an EQA scheme including this group of diseases. It is also desirable that the CPD is facilitated by membership of appropriate national societies (such as the British Society for Dermatopathology and/or the UK Cutaneous Lymphoma Group). Each cancer or pathology network could hold a panel of histopathologists suitable for SSMDT participation based on these criteria. Histopathologists restricting their activity in the SSMDT centre to work at the LSMDT level should be able to demonstrate the same activity as defined previously. It is acknowledged that because of workforce shortages in histopathology there could be an implementation delay of these goals in some centres." 4.2 The multi-disciplinary team meetings The following cancer cases should be reviewed by a cancer multi-disciplinary team locally (LSMDT), which has a Histopathologist as a core member. High risk/recurrent basal cell carcinomas or those involving margins All squamous cell carcinomas (optional) high risk/recurrent squamous cell carcinomas or those involving the margins (required) All melanomas All lymphomas All cutaneous sarcomas Rare skin tumours. Following this local review the following will be reviewed at the Network MDT (SSMDT). The IOG states that the following should be reviewed (there are more case types but they are clinically based criteria - see IOG): Complex BCC requiring Mohs or complex surgery High risk or metastatic SCC Newly diagnosed melanomas Stage 2B (2.01-4 mm with ulceration (T3bN0M0) or > 4 mm without ulceration (T4aN0M0) or higher, multiple melanomas and children under 19 years All lymphomas (these may be reviewed at oncology centre at the skin lymphoma MDT) All cutaneous sarcomas (these may be reviewed at the sarcoma MDT) Rare skin tumours. There should be a nominated lead and deputy pathologist for both the LSMDT and SSMDT. The histopathologists engaged in skin cancer diagnosis should participate in an appropriate external quality assessment (EQA) scheme and demonstrate evidence of continuing Page 41 of 70

professional development (CPD) relevant to skin cancer. The lead histopathologist should attend over 50% of MDT meetings.. Patients with lymphoma and other rare skin cancers should be dealt with by only one SSMDT in the network. All cases should be reviewed by the dermatopathologist designated by the network to have an interest in, and lead responsibility for, cutaneous lymphoma reporting. It is appropriate for the network lead dermatopathologist in lymphoma reporting to attend the clinics as necessary. The lead dermatopathologist in lymphoma reporting is likely to be, but is not necessarily, the SSMDT lead dermatopathologist. All cases referred to the SSMDT should receive formal diagnostic histopathological review. There may be a few exceptions; for example, cases referred with extensive BCC for surgical reconstruction. All cases requiring a tertiary histopathological opinion should be supported by the SSMDT on a commissioning basis. Specimens should be reported within an agreed time frame so as to allow appropriate clinical decision making at a planned MDT meeting. 4.3 Specimen Types Curettings, diagnostic punch biopsies, excisions. 4.4 Turnaround time in reporting Histopathology reporting time for suspected skin cancer in the NSSG should be within 14 calendar or 10 working days. Definitive reports for specimens sent for a second opinion may take longer but a report for preliminary clinical management should be issued. 4.5 Specimen examination The local protocol for specimen examination should take into account national guidelines and should be regularly reviewed and updated by the lead pathologists in consultation with other pathologists who participate in the service delivery. The minimum dataset recommends the following: "The margins of all skin biopsies undertaken for excisional purposes for melanocytic lesions and proven or suspected skin cancer should be marked using the standard technique of painting with substances such as silver nitrate, Indian ink, alcian blue or commercial preparations. When Mohs surgery has not been undertaken, this is the only way to obtain a reasonably accurate assessment and/or measurement of the peripheral and deep surgical margins. Its routine use with all large specimens, especially with clearly visible small central lesions, is more debatable. Even in these circumstances, however, the method remains desirable in view of the possibility of unexpected microscopic extension of the lesion. Curetted specimens, incisional biopsies, shave biopsies and punch biopsies (not performed for excisional reasons) do not necessarily require the margins to be marked. These specimens are often small and can be embedded in toto. Slightly larger pieces of tissue can be bisected. The examination of specimens requiring orientation can be facilitated by the Page 42 of 70

use of different coloured inks on different margins or the insertion of coloured agar into the cassette. The method of handling excisional biopsies depends on the size of the biopsy, whether the lesion can be seen, the position of the lesion on the specimen and the suspected type of cancer. It is recommended that separate judgements are made on each individual case taking these variables into account, together with the following guidelines. Small excisional biopsies (say up to 5 mm) can be embedded in toto. The requirement for steplevels/sections in any type of biopsy is dependent on the requirement to identify a lesion, establish a definitive diagnosis and assess the margins. There is, however, an increasing tendency to undertake step levels with severely dysplastic, in situ and difficult melanocytic lesions. In any larger specimen requiring trimming, in which the lesion can be seen, a basic principle is that the specimen should be cut at 2 5 mm intervals so that the nearest nakedeye margin to the lesion can be assessed histopathologically. For many skin ellipses, this will require transverse rather than longitudinal sectioning. When the lesions cannot be seen, transverse sectioning across the smallest diameter should be performed. When multiple sectioning is required, this should be undertaken by the sliced-bread / toast rack method. In specimens under 10 mm, it is recommended that all lesional tissue is examined or the entire specimen if the lesion cannot be seen. Excisional biopsies over 10 mm should take into account the type of skin cancer present. Generally, the whole of a suspected or proven malignant melanoma should be embedded and examined. For basal or squamous cell carcinoma, a sampling method is acceptable, taking blocks from areas of maximum lesional thickness, ulceration and the nearest margins. When the lesion is not discernible, the polar ends from the long axis of skin ellipses should be identified separately. These can be placed into one or two designated cassettes, depending on whether orientation of the specimen has been indicated clinically. Similarly, when the lesion can be seen, it is increasingly regarded as good practice to place the polar ends from the long axis of skin ellipses into one or two designated cassettes. Polar ends can be embedded and cut from the face down aspect. If initial sections show malignant involvement, step-levels can be undertaken to assess clearance up to the extreme peripheral margins. Cruciate margins at three, six, nine and 12 o clock can be sampled on larger circular specimens." 4.6 Grading and staging of skin tumours Basal cell carcinoma - the growth pattern should be reported according to the RCPath dataset. Squamous cell carcinoma - well, moderate, poorly and sarcomatoid. Melanoma - the subtype should be recorded according to the RCPath dataset. Page 43 of 70

Lymphoma - reported based on WHO classification. Sarcomas - reported based on WHO classification. Tumour staging: TNM classification of malignant tumours (6 edition). For melanomas the AJCC staging system: http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/dermatology/me lanoma/table1melanoma.htm 4.7 Use of ancillary laboratory techniques: All laboratories providing a pathology service in the network must have at least conditional CPA accreditation and ensure participation in an appropriate EQA programme, which demonstrates satisfactory laboratory performance. A wide range of immunohistochemical markers are available within the network. Those which are often used in the reporting of skin cancers include: S100, melan A, HMB45 - in melanomas Pan-cytokeratins - for carcinomas CD34, factor XIIIa in dermatofibrosarcoma pertruberans. 4.8 Audit All pathologists reporting skin cancer specimens should participate in a relevant EQA scheme and local audits (including an assessment of consistency where more than one pathologist participates in service provision). The audits should include: Review of compliance with procedures for specimen examination and reporting Completeness of minimum datasets Diagnostic agreement/disagreement during review of cases for MDTs Review of diagnostic consistency between pathologists using data from cases EQA circulation or blind circulations. The results of the audit process should be discussed with all pathologists who participate in service delivery. 4.9 Referral for review or specialist opinion NICE recommends that diagnostic biopsies are reviewed in the hospital where any definitive surgery is to be carried out. See also above regarding cases reviewed at SSMDT. Local specialists: Melanoma: Dr Keith Miller at NBT Lymphomas: Dr Pawade at UHB and Dr Rooney at NBT. Sarcomas: Dr Collins at UHB Tertiary referrals: Page 44 of 70

Dr Calonje at St Johns, London. Others include: Dr Jeff Theaker in Southhampton, Dr Nigel Kirkham in Newcastle, Dr Martin Cook at St Georges, Dr David Slater in Sheffield. 4.10 Additional staff costs The IOG states: Additional staff may need to be recruited to allow existing staff the time to attend meetings. Shortages of radiologists, pathologists and oncologists are likely to hinder the development and the ongoing operation of the MDTs. 1.75 histopathologists per network; annual employment costs of around 171,991" 4.11 TNM 7 The Royal College of Pathologists recommend using the AJCC 7th edition and not TNM7, [ http://www.rcpath.org/resources/pdf/adviceontmn7aug10.pdf ]. The RCPath datasets are due to be updated shortly. The datasets included in this document use the TNM6 classification. 4.12 Minimum dataset for reporting: These are based on the minimum dataset for histopathology reports as published by the Royal College of Pathologists. Skin Adnexal carcinomas and lymph nodes (Jul 2014) Basal cell carcinoma (May 2014) Malignant melanoma (May 2014) Merkel cell carcinoma (May 2014) Invasive squamous cell carcinoma (May 2014) 4.13 Enquiries All enquiries relating to this document should be addressed to: Keith Miller (keith.miller@nbt.nhs.uk) Consultant Pathologist, Southmead Hospital, North Bristol Trust, Westbury on Trym, BS10 5NB 5. Skin Cancer Surveillance and Management of Immuno-Suppressed Organ Transplant Recipients 5.1 Introduction There is very strong clinical and epidemiological evidence that patients who have had an organ transplant are at significant increasing risk of skin cancer, particularly SCC, but Page 45 of 70

melanoma and BCC too, as a result of long term immunosuppression. The IOG requirements for managing this group of patients, which also includes a small number of patients with an inherited tendency to skin cancer, will mean additional work and an increase in the numbers of follow up patients clinicians will have to seen. The NSSG has a dedicated skin cancer clinic for these high risk individuals, the most recent having started at North Bristol Trust in 2012 for the large cohort of renal transplant patients from this Trust. There are three main issues that need to be addressed: education, surveillance and treatment. The measure requires provision of service to immuno-compromised patients with skin cancer. Although this emphasises those with a diagnosis and their need for treatment and follow up, the Network is ambitious in setting out to define a service that will include education and early diagnosis consistent with the Cancer Reform Strategy. 5.2 Referral Pathway For Solid Organ Transplant and Immunosuppressed Patients Before Patient Develops Skin Cancer Transplant Clinic Patients should be given skin cancer prevention advice by clinical staff in transplant assessment clinics. Information must be backed up with written advice with specific information leaflets. Patients must be advised about the increased risk of skin cancer, the need for careful protection of the skin from significant UV exposure and need for prompt referral for persisting skin lesions via GP or transplant clinic. Education of what to look out for regarding changing lesions is important. Prior to discharge, post-transplant patients should be given further advice and education on skin cancer prevention and skin cancer recognition. An annual check of the skin should be undertaken in the transplant clinic. Any suspicious lesions should be referred to the local skin cancer service for assessment. 5.3 Patients With Suspected Skin Cancer If a patient develops a suspicious or persisting skin lesion, the patient must be referred by a 2 week wait pathway to a skin cancer clinic (either locally or in transplant centre). Transplant teams should refer electronically to the relevant skin cancer clinic (local or central by patient choice). GPs and GPwSIs should refer using the existing 2 week wait system. 5.4 Patients With Confirmed Skin Cancer If a skin cancer is diagnosed the patient should be treated as per the network guidelines for the cancer and should be discussed at the SSMDT. After the primary management of the skin cancer the patient should be followed up by the skin cancer service, and referred to the specialist skin cancer immunosuppression clinic if they have had a melanoma or two or more SCCs. Copy of the skin cancer diagnosis letter Page 46 of 70

should be sent to the GP and to the consultant in charge of on-going care for the transplant patient. 5.5 Patients With Multiple/Rare Skin Cancers Patients who develop two or more SCCs or a melanoma should be referred to the specialist immunosuppressed skin cancer clinic for consideration of further treatments, and discussion of the immuno-suppressive regime with the patient and the transplant team. Patients who develop skin lymphoma should be referred to the skin lymphoma MDT. Patients who develop skin cancers in specialist sites (e.g. perianal, oral) should be referred to the central clinic for discussion at the SSMDT and liaison with the relevant teams. Patients who wish to be seen at the central specialist clinic should be referred. Referral should be compliant with the policy for treatment of children and Teenagers and Young Adults. 5.6 Surveillance Annual skin examination to identify and treat malignant and pre-malignant lesions must be undertaken by the transplant centre or the local MDT. 5.7 Policy Patients may elect to have their annual surveillance undertaken either at the transplant centre or their local MDT. Specific clinics or clinic slots for this group of immunosuppressed individuals must be clearly identified on the MDT patient booking pattern and the numbers of these clinics or clinic slots adjusted to meet the demand. The clinic should have bookable numbered slots identified for immunocompromised patients. The doctor providing this service must be a core member of the MDT and this must be part of the doctor s job plan. Currently, Dr Amrit Darvay runs a monthly specialist clinic at NBT and Dr William Phillips has a clinic at the Royal United Hospital, Bath. There should be a regular clinic at the transplant centre where transplant recipients, without a history of skin cancer, can be reviewed annually by the CNS. This clinic should run alongside a skin cancer clinic staffed by a dermatologist who is a core member of the MDT so that a patient with a suspicious lesion can be treated, or booked in for treatment, on the same day. 5.8 Compliance At the transplant centre the number of surveillance clinics and the number of patients attending will be documented by the CNS and published annually. Page 47 of 70

The numbers of clinics identified for immunosuppressed patients must be published annually by the Lead Clinician for the named MDT in the annual report which must include clinic booking rules. Clinics must be shown to be part of consultants and CNS job plans. 5.9 Treatment It is recognised that a subset of patients, usually those who have had a longer history of immunosuppression, or of excessive sun exposure in the past, will require regular skin cancer surgery. 5.10 Policy All patients with a new lump that raises concern about the diagnosis of skin cancer should use the 2WW mechanism for referral. Patient and Renal physician education must help establish this. It is very important that there is continuity of care for this group of patients and that, where possible, they are seen by the same clinician on each visit. Usually this will be as a regular follow up patient seen every three months or so in the local MDT and undergoing surgery as required. The patient will have access to rapid review via the skin cancer nurse specialist. 5.11 Compliance Audit of this group of immunocompromised patients will be undertaken and presented annually at the MDT, and the Lead Clinician will include this in the MDT annual report. 5.12 Guidelines British Society for Skin Care in Immunosuppressed Individuals (BSCCII) The network supports the clinical guidelines agreed by BSCCII and several members of the Society are practicing skin cancer clinicians within our network. Details of the organisation can be found at: http://www.bsscii.org.uk/ The Transplant Skin Clinic Surveillance Visit form can be found here, and the Transplant Baselines Dermatology Risk Assessment form can be found here. 6. Oncology in Skin Cancer (Author Dr A Challapalli Oncology UHB) Summary of Treatment Guidance to Clinicians 6.1 SCC and BCC Treated With Primary Radiotherapy SCC <= 1cm or BCC<=2cm old frail patient single 18-20 Gy treatment BCC/SCC <= 3cm: 5 fractions in 1 week (alternative is 3 fractions in 3 weeks or 2 fractions 5 weeks apart) BCC/SCC 3-5cm: 8-15 fractions daily (generally 10) in 1 ½ -3 weeks BCC/SCC >5cm: 15-30 fractions daily in 3-6 weeks. Page 48 of 70

Length of course influenced by age/ site/ size/ frailty and can differ from the above if needed. Palliative treatments usually single. Options for Type of Radiotherapy: Electrons: Bristol and Bath. Need SCC and morphemic BCC>=1cm or BCC>=2cm, some difficulty over air (e.g. anterior nose field). Superficial X-ray (SXR): Taunton and Bath, limited tissue penetration- only useful if < 0.5/1cm deep, easiest to use near eyes as needs least lead to shield. Excellent for small lesions. Brachytherapy: Bristol or Cardiff, rarely used for complex fields (e.g. whole of scalp). Megavoltage Photons: Bristol and Bath, used if need deep tissue penetration. Patients to Avoid Radiotherapy: Gorlins (absolute) Xeroderma pigemtosa (absolute) Radiation induced skin ca (absolute) Ataxia telangectasia Fanconi anaemia?li-fraumani Syndrome Young - esp <50yr Middle of upper eyelid- however SXR can be excellent for edge of eyelid Limbs (esp hands and shins/ malleoli) Trunk- especially over spine Recurrence after prev RT Invades bone or significant cartilage involvement-surgery best if possible Site of previous burn Fragile tissue paper thin skin after steroids. 6.2 Joint Protocol for the Treatment of Skin Cancers with Radiotherapy Introduction Skin cancer should be managed by a multidisciplinary team including a dermatologist, clinical oncologist and plastic surgeon. Oncologists treating skin cancer should be members of the MDT. Surgery and radiotherapy are equally effective for treating basal cell and squamous carcinomas. Radiotherapy is usually preferred: Page 49 of 70

When the position of the carcinoma is such that the cosmetic result from surgery is likely to be poor or require extensive reconstruction It can be more appropriate for older patients Patients who are otherwise unfit for surgery Patients who refuse surgery Recurrence post surgery. Surgery is preferred: Younger patients (Especially <50years) When the carcinoma is on the trunk, extremities or upper eyelids Fixity to bone, cartilage or other deep structures Recurrence after radiotherapy Patients with Gorlins, xeroderma pigmentosa, ataxia telangectasia. 6.3 Indications for adjuvant Radiotherapy In the post-operative setting, adjuvant radiotherapy can reduce risk of relapse for primary carcinomas > 2cm, with perineural invasion and where there are positive/ close post operative margins. Lymph node metastases from carcinoma and melanoma are best treated surgically, but adjuvant radiotherapy can reduce the risk of relapse. Indications for this are discussed below. 6.4 Radiotherapy For Primary/Locally Recurrent BCC and SCC Basal cell and squamous carcinomas are treated with similar or identical dose and fractionation schedules. The schedule chosen is influenced by the size of the lesion and cosmetic considerations, not by the precise histology. More fractionated regimens are likely to produce better long term cosmetic results, particularly when volumes are large. They are also more suitable when there is underlying cartilage or bone, particularly for lesions on the pinna. Patients who have been treated surgically but who have positive or close margins or perineural invasion have a higher risk of local recurrence. These patients can be treated with further surgery, post-operative radiotherapy or occasionally a watch and wait policy. Risk factors for local recurrence in SCC (up to 25%) Site - lip, ear Size - tumours> 2cm Depth of invasion (> 4mm) Cellular differentiation Perineural involvement Host immune status and previous treatment Page 50 of 70

Tumours in non-sun-exposed sites, areas of previous radiation/ thermal injury/ scarring/ chronic ulceration Follow-up Basal Cell Carcinoma: Routine follow-up at 3-6 months following radiotherapy to confirm healing and eradication of neoplasm. Additional follow-up may be required in the initial few weeks after radiotherapy in sites where there is the potential for significant acute toxicity. Squamous Cell Carcinoma: Routine follow-up should adhere to local trust guidelines. Additional follow-up may be required in the initial few weeks after radiotherapy in sites where there is the potential for significant acute toxicity. Radiotherapy for SCC with Nodal Spread All patients with nodal disease will be discussed in the Specialist Skin Cancer MDT (currently held at Southmead Hospital and Musgrove Park Hospital). The majority will initially be treated surgically. Those at high risk of recurrence should be considered for post-operative radiotherapy to the nodal bed. Patients unsuitable for an operation will be considered for radical radiotherapy. Prior to this, all patients will be staged with a CT scan of the thorax and abdomen (and pelvis if inguinal node metastasis). Risk factors for post-operative recurrence include: Extra-capsular spread Positive nodal margin Nodes within the parotid gland involved Multiple nodes involved Large (>3cm) nodes involved. 6.5 Procedure for Post-Operative or Primary Radical Radiotherapy Follow-up Patients having head and/or neck irradiated: During radiotherapy patients will be reviewed weekly by the head and neck review radiographer. If the review radiographer is not available then the patient will be booked into a doctor review clinic for that week. The patients will be weighed weekly and if their weight drops or there is concern regarding their nutritional input then they will be reviewed by the dietician. If there is concern regarding aspiration then they will be reviewed by the speech and language therapist FBC and renal profile weekly Follow-up in clinic 2 weeks after completion of radiotherapy, then as clinically indicated until acute radiation reaction has settled. Then 3 monthly follow-up for 2 years, 4 monthly for 1 year then 6 monthly until 5 years follow-up is completed. Follow-up can be by oncologist, surgeon or joint. Patients having other nodal areas irradiated: Page 51 of 70

Weekly review in the doctor review clinic FBC and renal profile at start of treatment Follow-up in clinic at 6 weeks then as clinically indicated until acute radiation has settled. Then 3 monthly follow-up for 2 years, 4 monthly in year 3 then 6 monthly until 5 years follow-up is completed. Follow-up can be by oncologist, surgeon or joint. Procedure for Palliative Nodal Irradiation in SCC Location of the node, patient s performance status and presence of metastatic disease will affect choice of radiotherapy regime. Common palliative regimes include: Single 8Gy fraction 20Gy in 5# over 1 week 30-36Gy in 5-6# over 5-6 weeks 6.6 Procedure for Post-Operative Nodal Melanoma Adjuvant radiotherapy use in patients with nodal disease from melanoma is controversial. There are no reported randomised control trials in this area. Retrospective and historical data comparisons suggest that adjuvant radiotherapy reduces the risk of local recurrence, but does not alter overall survival due to the high rate of development of metastatic disease. Grade 3 lymphoedema will occur in 19% of patients after inguinal nodal basin and 9% after axillary nodal basin radiotherapy. Risk factors for local recurrence include: Parotid lymphadenopathy Extra-capsular spread >=4 involved nodes >=3cm diameter of largest node Recurrence after previous surgery Tumour spill at surgery or positive margins. 6.7 Procedure for Post-Operative Nodal Melanoma Radiotherapy Patients having head and/or neck irradiated: During radiotherapy patients will be reviewed weekly by the head and neck review radiographer. If the review radiographer is not available then the patient will be booked into a doctor review clinic for that week. The patients will be weighed weekly and if their weight drops or there is concern regarding their nutritional input then they will be reviewed by the dietician. If there is concern regarding aspiration then they will be reviewed by the speech and language therapist. FBC and renal profile weekly. Page 52 of 70

Follow-up in clinic 2 weeks after completion of radiotherapy, then as clinically indicated until acute radiation has settled. Then 3 monthly follow-up for 2 years, 4 monthly in year 3 then 6 monthly until 5 years follow-up is completed. Follow-up can be by oncologist, surgeon or joint. 6.8 Palliative Radiotherapy for Melanoma Depending on location of metastasis and patient s performance status radiotherapy may improve symptomatic metastases. Dose fractionation: 8-10Gy single fraction 20Gy in 5# in 1 week 30Gy in 10# in 2 weeks 30-36Gy in 5-6# over 5-6 weeks. Stereotactic radiosurgery may also be useful for up to 2 brain metastases. WBRT: 12 Gy/ 2#/ 2 days or 20Gy/ 5#/ 1 week. 6.9 Radiotherapy for Lentigo Maligna Surgery is the mainstay of treatment for lentigo maligna but radiotherapy may successfully treat this disease in patients who surgery would provide excessive cosmetic morbidity. Dose fractionation: 45Gy/ 10#/ 2 weeks, 50 Gy/ 20#/ 4 weeks 60 Gy/ 30#/ 6 weeks 6.10 Radiotherapy for Cutaneous Lymphoma All patients with a new diagnosis of skin lymphoma should be discussed in the SSMDT (Southmead, Bristol and MPH, Taunton) and those with rare cutaneous lymphoma, or advanced or resistant mycosis fungoides (MF), should be discussed in the supraregional meeting. Radiotherapy is appropriate treatment for localised disease and for symptomatic relief of more advanced disease. Some patients with advanced MF benefit from total skin electron therapy. At present this is provided at St. Thomas Hospital, London. Mycosis Fungoides Page 53 of 70

Patch: 8Gy in 2# in 1 week Plaque/ Tumour: 12Gy in 3# in 1 week Mucosal disease: 20Gy in 10# in 2 weeks Bulky Lymph Nodes: 15-30Gy in 5-15# in 1-3 weeks Alternative: 20Gy in 5# in 1 week Non-MF Cutaneous Lymphoma: Treatment for localised disease can be considered potentially curative. Dose depends on histology. PCMZL/ PCFCL 15Gy/ 5#/ 1 week PCLBCL 30Gy/ 15#/ 3 weeks Multiple indolent tumours or palliative intent: as per MF dose fractionation. 6.11 Merkel Cell Tumours Merkel Cell Tumours are rare and there are no randomised controlled trials to guide treatment. SEER database information suggests a significant improvement in survival with use of adjuvant radiotherapy by 18 months. Dose-fractionation: Radical: 60Gy/ 30#/ 6 weeks Adjuvant to tumour bed and prophylactic nodal irradiation 50Gy/ 25#/ 5 weeks. 45Gy/ 15#/ 3 weeks Palliation: 40Gy/ 10#/ 2 weeks, 30Gy/ 6#/ 1.5 weeks schedules are preferred to 30Gy/ 10#/ 2 weeks or 20Gy/ 5#/ 1 week. Consider Radiotherapy In: 6.12 Kaposi s Sarcoma Radiotherapy is used for palliation of symptoms and cosmesis. Skin lesions: 8Gy single fraction achieves good results or 15Gy/ 3#/ 1week. Mucosal lesions: 16Gy/ 8 #/ 1 ½ weeks, or 20Gy/ 10#/ 2weeks. 6.13 Cutaneous Angioosarcoma Tumours are ill-defined and often multifocal Common on scalp and face Page 54 of 70

Aggressive with high risk of mets - 5 yr survival is 15% with a 50% mortality rate at 15 months Primary treatment is surgery if possible Adjuvant radiotherapy for involved or close margins. Dose fractionation: Radical: 55Gy/ 20#/ 4 weeks, 60Gy/ 30#/ 6 weeks Adjuvant: 50Gy/ 25#/ 5 weeks Palliative: 44Gy/ 11#/ 3.5 weeks. 6.14 Keloids Reduce risk of recurrence after surgery. CTV=Scar + 0.5cm. Dose fractionation: 14Gy/ 2 #/ 1 week apart. First dose within 24hours of surgery 10Gy/ SF within 24 hrs of surgery. 6.15 Skin Metastasis For palliation of pain, bleeding, cosmesis. 8 Gy in 1 fraction if poor performance status/limited prognosis. 20 Gy in 5# if good performance status/longer prognosis. 6.16 Options for Type of Radiotherapy: Electrons: Bristol and Bath. For SCC and morphoeic BCC>=1cm or BCC>=2cm, some difficulty over air (e.g. anterior nose field). Superficial X-ray (SXR): Taunton and Bath, limited tissue penetration- only useful if < 0.5/1cm deep, easiest to use near eyes as needs least lead to shield. Excellent for small lesions. Brachytherapy: Bristol or Cardiff, rarely used for complex fields (e.g. whole of scalp). Megavoltage Photons: Bristol and Bath, used if need deep tissue penetration. 7. Clinical Management of Rare Skin Tumours Including Skin Lymphoma 7.1 Introduction Page 55 of 70

The management of rare tumours in the NSSG requires close collaboration between separate MDTs, where the LSMDT is the usual entry point for diagnosis. Tertiary referral may involve a combination of the SSMDT at Southmead and other anatomical site-specific groups. Guidelines on the management of these tumours seldom has a strong evidence base due to the relatively small numbers. Accordingly, there are seldom UK national guidelines and examples are drawn from the National Comprehensive Cancer Network in the USA. This in turn leads to conservative arrangements for follow up that err on the side of keeping the patient monitored in secondary care. In terms of general skin cancer activity, the numbers are small. 7.2 Dermatofibromasarcoma Protuberans: 7.2.1 Clinical Behaviour DFSP is a low grade sarcoma probably of fibroblast origin. Thought to arise in the dermis and invade deeply. Population incidence is approx 1 case per 4 million per annum. Complex, irregular 3D tumour structure explains high local recurrence rates. The risk of regional spread is approx 1% and metastatic spread 5%. DFSP is slow growing. Diagnosis is often delayed. It may start as a papule gradually enlarging into a nodule or plaque. Colour varies from pink to red to violaceous. Plaques may ulcerate. DFSP most commonly occurs on the trunk, followed by the proximal extremities. It rarely occurs above the neck. 7.2.2. Diagnosis and Investigation Suspicious lesions should be referred to the SSMDT. Initial deep incisional biopsy is recommended. Equivocal histology may require further biopsy. Imaging is not recommended as the risk of metastasis is low. 7.2.3 Level of Care Suspected or biopsy proven DFSP above superficial fascia should be referred to the SSMDT. Below the fascia Sarcoma MDT should be involved with decision making. 7.2.4 Treatment Resection is the first line treatment, margins can be controversial. Most authorities recommend a 3 cm clearance, down to and including fascia, if possible. Five year recurrence free survival rates increased with increasing margins of resection: 59 % less than 1 centimetre clearance 66 % greater than or equal to 1 centimetre clearance 70 % greater than or equal to 2 centimetre clearance 80 % greater than or equal to 3 centimetre clearance. A 4cm macroscopic margin is said to give clearance of 95% for DFSP. Page 56 of 70

Mohs surgery may further improve outcome (local recurrence rate 6.6%) vs. wide excision (local recurrence rate 10%). If there is doubt about resection margins, split skin graft will allow monitoring of the bed. Additional treatments: 7.2.5 Radiotherapy Should be considered when complete excision is not obtainable or when resection of metastatic disease is not feasible. 7.2.6 Chemotherapy Imatinib mesylate (Gleevec) is FDA approved for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. Since this is a rare indication, treatment should be initiated by an oncologist and funding would need to be agreed. 7.2.7 Follow-up: Year 1 3 monthly review. Year 2 3 monthly review. Year 3 3 monthly review. Year 4 6 monthly review. Year 5 6 monthly review. Consider annual review thereafter. A history & examination for local recurrence and regional lymphadenopathy is appropriate but further investigation is not warranted unless metastatic disease (lung is the most common) is suspected. Because of the indolent nature of DFSP the patient should be coached regarding lifelong surveillance. References:www.nccn.org/professionals/physician_gls/PDF/dfsp.pdf 7.3 Atypical Fibroxanthoma: 7.3.1 Clinical Most authorities would hold that atypical fibroxanthoma (AFX) is a dermal lesion with a favourable prognosis. It most commonly occurs on the face, nose, ears & scalp of elderly individuals and may be linked to ultraviolet radiation. It is more frequent in males (70% men; 30% women), average age 70. AFX is a nodular or plaque like erythematous or tan coloured dermal lesion which typically shows rapid growth. Lesions are usually less than 2 cm in diameter and half the cases are ulcerated at presentation. It is also reported in younger individuals in non exposed sites of trunk and Page 57 of 70

limbs and also immune suppressed transplant patients. Currently there is debate as to whether it is a benign or malignant tumour. It has been reported to metastasise but there is argument that these are cases of pleomorphic malignant fibrous histiocytoma (MFH). 7.3.2 Pathology Most AFX cases (80%) are restricted to the reticular dermis. The lesion may extend into the upper one-third of the subcutaneous adipose tissue. Tumours that extensively involve subcutis, penetrate fascia, or display necrosis, perineural or lymphovascular invasion should be diagnosed as MFH. Cell types include spindle, epithelioid, multinucleate and bizarre cells, in various proportions, arranged in haphazard, vaguely fascicular or storiform patterns with hyperchromatic and multilobulated nuclei. 7.3.3 Treatment There are no widely agreed recommendations for the treatment of AFX. In the absence of clear guidelines a 10mm clearance in the first instance would be suggested. If this does not obtain clearance wider excision would be recommended; Moh s surgery should be considered. Levels of Care Cases of AFX should be treated as for high risk SCC patients as per the peer review measures. 7.3.4 Follow-up There are no widely agreed recommendations for follow-up. In the absence of clear guidelines: Year 1 3 monthly review. Year 2 3 monthly review. Year 3 3 monthly review. Year 4 6 monthly review. Year 5 6 monthly review. References: Atypical Fibroxanthoma A.SAKAMOTO.Clinical Medicine.Oncology 2008; 2: 1192129 7.4 Kaposi s Sarcoma: 7.4.1 Introduction Page 58 of 70

Kaposi's sarcoma (KS) is a tumour caused by Human Herpes Virus 8 (HHV8). Although there are different clinical subtypes, HHV-8 is the common aetiological cause. Not considered a true sarcoma it arises from malignant transformation in lymphatic endothelium. 7.4.2 Classic Kaposi s Sarcoma Typically affects elderly men of Mediterranean or Eastern European descent where HHV-8 infection is relatively high. 7.4.3 Endemic Kaposi s Sarcoma Typically affects young sub-saharan Africans. Unrelated to HIV it is more aggressive than classic KS. The most common presentation is with extensive infiltration of skin, especially lower limbs. 7.4.4 Transplant Related Kaposi s Sarcoma Occurs in relation to suppressed T-cell function when an HHV 8-infected organ is transplanted or when the recipient already has pre-existing HHV 8 infection. 7.4.5 Epidemic Kaposi s Sarcoma Is AIDS-related in patients with suppressed T-cell function where HHV 8 is usually sexually transmitted. 7.4.6 Level of Care Optimal care can only be achieved by the close co-operation of oncologists, haematologists and HIV physicians. Professor Woll at Weston Park Hospital has a particular interest in Kaposi Sarcoma. 7.4.7 Clinical Features Kaposi s Sarcoma lesions are nodular or plaques that may be red, purple, brown, or black. Tumours are vascular and extravasation into surrounding tissues produces its discolouration. Inflammation produces pain and swelling. Lesions are most commonly found on the skin but may occur in gastrointestinal or respiratory mucosa. Rate of progression and mortality varies widely. 7.4.8 Diagnosis Clinical features and index of suspicion must be confirmed by biopsy. Histological features and the detection of the KSHV protein LANA-1 (latency associated nuclear antigen) in tumour cells confirms the diagnosis. 7.4.9 Staging The AIDS Clinical Trials Group (ACTG) 1988 proposed a staging classification for AIDS-related Kaposi sarcoma (KS) that considers 3 criteria: Page 59 of 70

The extent of the tumour (T) The status of the immune system (I), as measured by the CD4 count The extent of involvement within the body or systemic illness (S). Under each of these major headings, there are 2 subgroups identified by either a zero (0) or a one (1). A zero (0) means good risk, while a one (1) means poor risk. 7.4.10 T (tumour) status T0 (good risk): Localized tumour. KS is confined to the skin and/or the lymph nodes. If there are lesions in the mouth, they can only be on the palate (roof of the mouth), and those lesions are flat (not raised or rounded). T1 (poor risk): Disseminated (widespread) tumour. One or more of the following is present: Oedema due to the tumour Lesions are ulcerated Extensive oral KS (nodular lesions and/or lesions not limited to the palate) KS is affecting the stomach or intestines KS is in the lungs or other internal organs. I (immune system) status I0 (good risk): CD4 cell count is at least 200 cells/ microliter. (Normal range 600-1,500). Some experts use a lower cut-off for good risk such as 150 or 100. I1 (poor risk): CD4 count is lower than 200 (again some have used lower cut-offs such as 150 or 100). S (systemic illness) status S0 (good risk): No systemic illness present and all of the following are true: No history of opportunistic infections or thrush (a fungal infection in the mouth) No B symptoms are present. B symptoms are: Unexplained fever Night sweats Weight loss of more than 10% (without trying to lose weight) Page 60 of 70

Diarrhoea for more than 2 weeks The patient is doing well - up and about most of the time and he can take care of himself. Karnofsky performance status needs to be at least 70 to be in the good risk category. S1 (poor risk) Systemic illness is present; and one or more of the following is true: History of opportunistic infections or thrush One or more B symptoms is present Performance status score under 70 Other HIV-related illness is present, such as neurological (nervous system) disease or lymphoma. Survival T and S factors seemed to be the most important. Ninety percent of KS patients who are at good risk in these categories (T0S0) survive for at least 3 years after diagnosis. Only fifty percent of the KS patients who are at poor risk in both of these categories (T1S1) are still alive 3 years after diagnosis. The immune system status when the patient is first diagnosed seems to be less important if HAART is given. 7.4.11 Treatment Kaposi's sarcoma is not currently curable. Treatment is palliative. In KS associated with immunodeficiency or immunosuppression, treating the immune dysfunction can slow or halt progression of KS. In 40% or more of patients with AIDS-associated Kaposi's sarcoma, the Kaposi lesions will shrink upon first starting highly active antiretroviral therapy (HAART). However, in a certain percentage of such patients, Kaposi's sarcoma may again grow after a number of years on HAART, especially if HIV is not completely suppressed. Patients with a few local lesions can often be treated with local measures such as radiation therapy or cryosurgery. Surgery is generally not recommended as Kaposi's sarcoma can appear in wound edges. British HIV Association guidelines for HIV-associated malignancies 2008 make the following recommendations: Early-stage KS (T0 stage) HAART. Consider local radiotherapy or liposomal anthracycline for rapidly progressing or cosmetically disfiguring disease. Advanced-stage KS (T1 stage) HAART and liposomal anthracycline (either DaunoXome 40 mg/m2 every 14 days or Caelyx 20 mg/m2 every 21 days) Page 61 of 70

Anthracycline-refractory KS HAART and paclitaxel (100 mg/m2 every 14 days) (individual case basis). References: British HIV Association Guidelines for HIV Associated Malignancies 2008 M Bower, S Collins, C Cottrill, K Cwynarski, S Montoto, M Nelson, N Nwokolo, T Powles, J Stebbing, N Wales, and A Webb. HIV Medicine 2008; 9: 362-388. 7.5 Merkel Cell Carcinoma Key Points Merkel cell carcinoma (MCC) is a rare and aggressive form of skin cancer that can grow rapidly and metastasise (spread) to regional lymph nodes and/or other distant sites. MCC often presents as a smooth, painless, firm lump located in, or just beneath, the skin. It can appear pink, blue or the same colour as the surrounding normal skin, and most commonly occurs in sun-exposed skin. Any patient who presents with a lesion that has features suspicious for MCC should be referred urgently for assessment by a core member of the Specialist Skin Multi- Disciplinary Team (SSMDT). Any patient found to have a histological diagnosis of MCC following biopsy of a lesion should be referred urgently for assessment by a core member (dermatologist or plastic surgeon) of the SSMDT. 7.5.1 Introduction MCC is a rare form of skin cancer that is thought to arise within neuroendocrine cells in the skin. Hence, it is sometimes known as primary cutaneous neuroendocrine carcinoma. MCC mainly affects adults over the age of 50 years (95% of cases) and is more common in white males than in other groups. It usually arises in sun-exposed skin, presenting as a painless, smooth lump that is firm and appears within or just beneath the skin. The appearance can vary in colour from pink to blue to normal skin colour. It is an aggressive cancer that can grow rapidly, recur locally following excision and metastasise to regional lymph nodes and/or distant sites, such as the lungs, liver and brain. It is because of these features that the management of all patients with MCC should be carried under the guidance of a SSMDT. 7.5.2 Level of Care Any patient who presents with a lesion that has the appearance of MCC should be referred urgently to a core member of the SSMDT who is able to confirm the diagnosis and initiate appropriate treatment (dermatologist or plastic surgeon). Any patient who has undergone biopsy of a lesion that is subsequently found to be an MCC should be referred urgently to a core member of the SSMDT who is able to perform a wide local excision (plastic surgeon) or Page 62 of 70

Mohs surgery (dermatologist). Any patient who presents with metastatic MCC should be referred for discussion at the SSMDT meeting and assessment by a core member of the SSMDT (oncologist, plastic surgeon, dermatologist). 7.5.3 Management There are currently no UK national guidelines for the management of MCC. Guidelines have been produced for MCC in the USA by the National Comprehensive Cancer Network, as part of the NCCN Clinical Practice Guidelines in Oncology (www.nccn.org). In general, the evidence base for these guidelines consists of lower level studies rather than randomised controlled trials and meta-analyses. The recommendations contained within the present guidelines arose from interpretation of such existing guidelines within the context of current regional practices and available resources. The guidelines are intended to illustrate the standard practice for the management of MCC within Avon, Somerset and Wiltshire region. However, it is acknowledged that there will be occasions when it is appropriate for the management of a patient to follow a course that differs from that outlined in these guidelines. This should generally only occur following consideration of the case by the SSMDT. 7.5.4 Investigation Primary disease: Patients presenting with primary lesion suspicious for MCC or following diagnostic biopsy of primary lesion. Perform diagnostic biopsy (preferably excision biopsy) if not already performed to obtain histological diagnosis. Histology slides from biopsies performed outside the remit of the SSMDT should be forwarded for review by a consultant histopathologist who is a core member of the SSMDT. In addition to routine haematoxylin and eosin (H&E) staining, histological analysis should involve immunohistochemistry with an immunopanel preferably containing cytokeratin-20 (CK-20) and thyroid transcription factor 1 (TTF-1), to differentiate MCC from metastatic small cell lung carcinoma, the histological appearance of which can be very similar. Patients should undergo clinical examination of the entire skin to look for concurrent lesions. Clinical examination of regional and distant lymph node basins should be performed to detect nodal metastases and abdominal examination for masses and/or hepatosplenomegaly should be carried out. Liver function tests should be performed as a screening test for liver metastases and if significantly deranged, radiological imaging of the liver should be performed. Further imaging modalities such as plain chest radiography, computed tomography, ultrasound and positron emission topography should only be performed as clinically indicated. If the CK-20 is negative and/or there is clinical suspicion that the lesion may not be a primary tumour, investigations should be considered to evaluate for a non-cutaneous primary carcinoma, such as small cell lung carcinoma. Page 63 of 70

If the lesion is believed to be a primary MCC and there is no evidence of metastases treatment should follow the guidelines for Stage I disease. Local recurrence and in transit metastases: Patients presenting with possible or confirmed metastatic nodal disease. Obtain, confirm or review diagnosis of metastatic nodal MCC. Patients with a previous history of MCC who present with a possible recurrence should be referred to the SSMDT or to an appropriate core member who is capable of confirming the diagnosis. Histology slides from biopsies performed outside the remit of the SSMDT should be forwarded for review by a consultant histopathologist who is a core member of the SSMDT. A clinical examination should be carried out as for patients presenting with a primary lesion (see above). If there is no previous history of MCC, particular attention should be directed towards detecting a primary lesion. FNA (fine needle aspiration) with or without ultrasound imaging/guidance would normally be performed on the clinically suspicious lymph node. Histological analysis of the FNA should include an immunopanel containing CK-20 and pancytokeratins. If FNA is negative an open excision biopsy of the lymph node should be performed. If FNA is positive then further staging investigations may be appropriate to assess the extent of nodal involvement and/or detect distant metastases. Once the diagnosis has been confirmed, distant metastasis has been excluded by staging investigations as clinically indicated, and the case has been referred for discussion at the SSMDT meeting, treatment should follow the guidelines for Stage II disease. 7.5.5 Metastatic Disease Patients presenting with possible or confirmed distant metastatic disease: Obtain, confirm or review diagnosis of metastatic MCC. Patients with a previous history of MCC who present with a possible distant metastasis should be referred to the SSMDT or to an appropriate core member who is capable of confirming the diagnosis. Histology slides from biopsies performed outside the remit of the SSMDT should be forwarded for review by a consultant histopathologist who is a core member of the SSMDT. A clinical examination should be carried out as for patients presenting with a primary lesion (see above). Particular attention should be directed towards other possible sites of distant metastasis, as guided by the clinical history. Appropriate radiological studies should be performed as indicated to aid diagnosis and plan treatment. Histological confirmation of the diagnosis may be possible but in some cases this may not be appropriate. 7.5.6 Treatment Stage 1 Disease: Excision of Primary Tumour Page 64 of 70

Treatment aims to achieve complete excision of the primary tumour demonstrated histologically by clear surgical margins. A wide local excision of the primary tumour with margins 2.5cm down to the investing fascia of the muscle or to the pericranium is recommended where possible. Excision with margins 0.5cm has been shown to be associated with a very high recurrence rate (100%). The local recurrence rate with margins 2.5cm appears to be reduced but still remains substantial (49%). The role for Mohs micrographic surgery for primary MCC excision remains unclear. One small study (12 patients) found the rate of recurrence to be similar to that of wide local excision (50%). However, in cases where the tumour is in an anatomically sensitive location Mohs surgery should be considered as an alternative to wide local excision. Reconstruction of defect following excision of primary tumour The choice of reconstruction for the defect resulting from wide local excision will be influenced by a variety of factors including the level of certainty that excision is complete, the general health of the patient and cosmetic concerns. Primary closure will usually be performed if possible. When primary closure is not possible a split skin graft should be considered as an option which provides thin skin cover and thus facilitates monitoring for local recurrence. If reconstruction using local or distant flaps is to be performed it is recommended that the procedure be delayed until histological confirmation of clear surgical margins has been received. Adjuvant Therapy For small tumours that are completely excised, and in the absence of other adverse risk factors, observation only should be considered. Radiotherapy to the primary tumour site should be considered for large tumours, tumours with microscopically involved margins following wide local excision or macroscopically involved margins where complete excision is not possible. Radiotherapy to in-transit lymphatics and draining nodal basins should be considered for head and neck primary tumour sites. There are currently no indications for chemotherapy for Stage I disease. Stage II Disease Treatment options should be discussed at SSMDT and include node dissection, radiotherapy or both. Chemotherapy has not been shown to improve disease-free or overall survival and is therefore not recommended outside clinical trial situations. Stage III Disease Treatment options should be discussed at SSMDT, should be tailored for the individual patient and may include best supportive care with or without surgery, radiotherapy and chemotherapy alone or in combination. Page 65 of 70

7.5.7 Follow-up The recommended follow-up schedule is the same for all patients with MCC irrespective of the stage of their disease. Year 1 3 monthly review. Year 2 3 monthly review. Year 3 3 monthly review. Year 4 6 monthly review. Year 5 6 monthly review. Consider annual review thereafter. References: www.nccn.org/professional/physician_gls/pdf/mcc.pdf Guidelines reviewed and re written by Dr Challapalli Consultant Oncologist BHOC UHB Sept 2014 7.6 Clinical Management of Cutaneous Lymphoma 7.6.1 Local Skin Cancer MDT All new cases of Cutaneous Lymphoma to be referred from LSMDT (care level 4) to SSMDT (Care level 5). Skin lymphoma SSMDT at Bristol Oncology Centre, University Hospitals Bristol NHS Foundation Trust is comprised of a Haemato-oncologist, Clinical Oncologist, Medical Oncologist, Specialist Lymphoma Histopathologist, a Dermatologist with specialist expertise in phototherapy, Haemato-Oncologist nurse and an MDT co-ordinator. 7.6.2 Referral Pathways: Clinical referral (Management referral of special groups) Referral pathways of these patients into the SSMDT will be via the agreed pro-forma. It is helpful to include sufficient clinical information and possible digital photographs. And/or Pathology referral Where the histology is uncertain, the pathology can be referred to the SSMDT for review. Dr Joya Pawade is the present named histopathology lead for cutaneous lymphoma for the Network and this work is carried out under her leadership. Specialist Skin Cancer MDT Page 66 of 70

Bristol Oncology Centre University Hospitals Bristol Foundation Trust LSMDT is the only named MDT to manage cutaneous lymphoma within the SWAG region. All LSMDTs can refer their patients for level 5 care to the Skin MDT in Bristol. The NSSG agrees that all cases should be reviewed by the dermatopathologist designated by the Network to have an interest and lead responsibility for cutaneous lymphoma reporting. All lymphoma patients should undergo diagnostic biopsies for histology, immunophenotyping and molecular studies, and this should be correlated with clinical presentation for accurate diagnosis and prognosis. It is recommended that the WHO-EORTC primary cutaneous lymphoma classification should be used to classify cases. Joint British Association of Dermatologists and UK Cutaneous Lymphoma Group guidelines for the management of primary cutaneous lymphomas. Whittaker SJ et al. Brit J Derm 2003; 149:1095-1107. 7.6.3 Management The IOG states (page 119) that all patients should be seen by and managed by the SSMDT. Such a transfer of care may not be in the patient s interest for routine management of less complex skin lymphoma, especially where they live at a distance from Bristol. When cases are referred to the SSMDT for histopathology review, clinical review and management advice, there will be flexibility on whether they are referred back to LSMDT for management, managed by clinicians, within the SSMDT or referred to the supranetwork MDT for TSEB or photophoresis. LSMDTs should be involved once the diagnosis and staging has been confirmed by the SSMDT. Patients with lymphomatoid papulosis and stage 1a mycosis fungoides could be managed locally by the LSMDT after diagnosis. Initial staging imaging is required on all patients with the exception of stage 1 mycosis fungoides and lymphomatoid papulosis. Patients with early stage MF (1B-11A) who are resistant to skin directed therapies should be reviewed by the supranetwork centre as at risk of disease progression. 7.6.4 Haemato-oncology Links The Skin Lymphoma SSMDT is an integrated haemato-oncology and Dermatology MDT. 7.6.5 Referral Pathway Patients regarded by the Network SSMDT to require supranetwork SSMDT referral will be referred to Supranetwork service at St Thomas s Hospital in London. In due course the SSMDT in Bristol would like to provide this service. 7.6.6 Supranetwork MDT for Total Skin Electron Beam Therapy Page 67 of 70

The Network Board should, in consultation with the skin cancer NSSG and SSMDT, agree with the specialist commissioners a guideline which specifies: Cases of nodular mycosis fungoides (stage 2b or over) should be referred for discussion and consideration of TSEB The named supranetwork MDT that the Network uses for this function is at St Thomas s Hospital, London. 7.6.7 Photophoresis Cases of erythrodermic cutaneous T-cell lymphoma, stages 3 and 4, having both skin involvement and circulating T-cell clonal cells should be discussed at the SSMDT. Potential cases will be discussed at the Network SSMDT and then referred to Bristol Oncology centre for consideration of photophoresis. (contact Dr Giles Dunnill on giles.dunnill@uhbristol.nhs.uk 7.7 Clinical Management of Palpable Lymph Nodes in Someone with a Previous Diagnosis of Skin Cancer 7.7.1 Protocol for Assessment of Palpable Lymph Nodes in Someone with a Previous Diagnosis of Skin Cancer These patients present having found a lump in the axilla, groin or neck or parotid themselves or a lump is detected on examination during follow-up. Note in accordance with NICE Improving Outcomes Guidance, such patients need to be referred at this point to the Skin Specialist MDT. Fine needle aspiration cytology (FNA) should be performed if there is clinical suspicion that such a lump represents metastatic spread. If the lump is highly suspicious, particularly in the head and neck/parotid, an MRI scan is requested at this stage, the patient is counselled for the node dissection and a provisional operating slot is identified. If this yields a positive result confirming spread of the skin cancer then the patient will usually be offered staging to determine the presence of any distant spread, prior to lymph node dissection of the appropriate lymph node basin. Page 68 of 70

If initial FNA yields insufficient material for cytological assessment or is in some other way inconclusive then usually FNA should be repeated, with ultrasound guidance if the procedure was technically difficult first time. If initial FNA yields lymphocytes, but without evidence of malignancy, and clinical suspicion remains, or if FNA was performed a second time still with an inconclusive result, then either core biopsy or more usually open lymph node biopsy should be undertaken. If open biopsy is undertaken, it is important that the incision be placed in the line of the subsequent completion lymphadenectomy incision. For highly suspicious nodes (on clinical and radiological assessment) the patient can be booked directly for open biopsy, intra-operative frozen section/cytology and completion node dissection if positive. Open biopsy yielding a positive result triggers restaging before proceeding to node dissection if appropriate, as in step 2. A non-positive result leads to reassurance or (for example in lymphoma) re-direction to appropriate MDT (e.g. haematology). Staging takes the form of chest X-ray and liver ultrasound or CT scan of chest / abdomen / pelvis. Commonly, if CT is used, views of the relevant nodal basin are included for surgical planning. If a mass is confirmed as a lymph node containing metastatic skin cancer, but without a prior known primary tumour, then further investigation is warranted. This should include clinical examination of the relevant anatomical area and if in the head and neck, ENT opinion, pan-endoscopy and ophthalmic assessment. If nothing is revealed, PET CT scan is then indicated. 7.7.2 The NSSG guide to Management of Palpable node Flow Chart The Management of palpable node flow chart can be found here. Guidelines re written by Mr Dominic Ayres, Consultant Plastic Surgeon NBT, Sept 2014 8. Appendix 1 Page 69 of 70

Enquiries All enquiries relating to this document should be addressed to: Dr Amrit Darvay Dept of Dermatology Southmead Hospital. Amrit.darvay@nbt.nhs.uk -END- Page 70 of 70