Cancer of Unknown Primary (CUP) Protocol

1 Department of Oncology. Cancer of Unknown Primary (CUP) Protocol Version: Document type: Document sponsor Designation Document author [ s] Designation[s] Approving committee / Group Ratified by: Date ratified October 2014 Date active from October 2014 V003 Clinical Protocol Nick Gallegos Medical Director Dr Thomas Wells / Corrine Thomas Consultant Oncologist / Lead Oncology Nurse Cancer Leads Group Cancer Leads Group Review date April 2016 Target audience: All clinical staff. If you require this document in a different format, please telephone the Patient Advice and Liaison Service (PALS). Please see local information for contact details. 1

2 Contents Introduction Who and when to contact for advice Roles and Responsibilities Aim Important Definitions 3 broad groups of CUP patients Patient Groups not to miss: patients with a potentially better prognosis Patients with disease requiring URGENT specific action Basic assessment Further investigations Biopsy Which Multidisciplinary team Meeting (MDT) should patients be discussed in? References Weston Area Health Trust (WHAT) follows the UKONS Acute Oncology Initial management Guidelines, 2013 for cancer of Unknown Primary management pathway [CUP] Guideline 21 page 33: http://www.aswcs.nhs.uk/mediafiles/downloads/65331673/acute_oncology_initial_managment_ Guidelines.pdf 2

3 Introduction: Patients diagnosed with Cancer of Unknown Primary (CUP) often present as emergencies (72% of such cases presented acutely between1997-2007). Length of stay can often be prolonged by lack of specialist review and inappropriate investigations. There is often a delay in identifying these patients and referring them for specialist review, this may well be influenced at WGH by the introduction of Acute Oncology Service (AOS). As of March 2014, the WAHT CUP service links into the network CUP/ MUO MDT which takes place by video conferencing via Bristol on a Thursday morning in the hospital academy. As the numbers are small, the AOS can coordinate the pathway and support these patients as nurse key workers in conjunction with input from the Palliative Care Team and other healthcare professionals within Weston Area Healthcare Trust (WAHT). Data collection and audit on this group of patients will aid future planning, and one of the main challenges will be the impact on existing oncology and specialist palliative care services. Hospital patients should be reviewed by a member of the CUP/AOS team by the end of the next working day after referral, or within 2 weeks as an outpatient. GPs can phone for advice at designated times (please see below) Who and when to contact for advice: HOSPITAL PATIENTS WITH SUSPECTED CUP: SHOULD BE REFERRED TO THE AOS FOR ADVICE; PLEASE SEE LOCAL INFORMATION FOR DETAILS. GPs: CAN PHONE THE AOS CLINICAL NURSE SPECIALIST FOR ADVICE / INFORMATION; PLEASE SEE LOCAL INFORMATION FOR DETAILS. PLEASE PERFORM FULL HISTORY AND EXAMINATION AND BASIC ASSESSMENT / TESTS (AS INDICATED IN THIS PROTOCOL) BEFORE PHONING FOR ADVICE. Roles and Responsibilities: Please see local information for details of the Lead Clinician for Management of Patients with CUP and the AOS / CUP Clinical Nurse Specialists. The Acute Oncology Nurses can help to facilitate the patient s pathway by: Contributing to multidisciplinary discussions and care planning Providing expert nursing advice and support for health professionals looking after Oncology & Haematology patients in a general setting. Communicating and supporting patients and carers during potentially distressing situations such as breaking bad news. Ensuring that the patients holistic needs assessments are taken into account during decision making process. 3

4 Aim: The aim of the pathway is to enable early identification of patients that would benefit from anticancer treatment and to prevent unnecessary investigations in those unfit for treatment. Important Definitions: Malignancy of undefined primary origin (MUO): this is metastatic malignancy identified after a limited number of tests, without an obvious primary site. Provisional carcinoma of unknown primary (CUP): metastatic epithelial or neuro-endocrine malignancy on the basis of biopsy, with no primary site identified despite initial investigations and before specialist review. Confirmed CUP: as above, but after specialist review and appropriate specialised investigations. 3 broad groups of CUP patients to be aware of: 1. A group of patients with a potentially better prognosis. 2. Patients in which appropriate investigations will aid palliative treatment. 3. Those in which further investigations would not alter prognosis or management, but may increase morbidity. Patient Groups not to miss: patients with a potentially better prognosis: Adenocarcinoma in the axillary nodes in women - treat as breast cancer. Young men with testicular masses, or mediastinal /retroperitoneal masses of germ cell origin. Women with peritoneal disease/ovarian mass who may respond well to ovarian cancer chemotherapy regimes. Patients with squamous cell carcinoma in cervical lymph nodes- possibility of radical head and neck cancer treatment. Patients with squamous cell carcinoma of the inguinal nodes, if no primary is identified, may be candidates for surgery. Patients who may have a colorectal primary. Those with solitary lesions. Patients with disease requiring URGENT specific action: Spinal cord compression requires urgent admission and referral to the spinal cord compression co-ordinator. Men with midline disease require urgent referral to oncology team specialising in germ cell tumours. Superior vena cava obstruction require urgent referral to lung MDT to discuss options for treatment (stent, radiotherapy, chemotherapy). Suspected lymphoma, myeloma, plasmacytoma require urgent referral to haematology. 4

5 Basic assessment / tests: Complete medical history including history of previous malignancy. Physical examination including breasts; thyroid; nodes; skin; testes; rectum; prostate; pelvic examination. Blood tests: FBC, U and Es, Creatinine, LFTs, Calcium, LDH, CRP, Glucose, TSH AFP if hepato-cellular cancer suspected. PSA if prostate cancer suspected. AFP, β-hcg and LDH if germ cell tumours suspected e.g. testicular masses or mediastinal/retroperitoneal masses in young men. CA125 in women with peritoneal disease, ovarian masses, ascites, pleural effusions or inguinal nodes. CEA, CA15-3 and CA19-9 are not specific or sensitive enough in this setting and should not be requested. Urinalysis Further investigations: Myeloma screen if lytic bone lesions. CXR CT scan chest, abdomen and pelvis. Please note: Upper or lower GI endoscopy is ONLY indicated if patients have upper or lower GI symptoms, or CT scan indicates a primary at these sites. Mammography is only indicated if a breast primary is suspected. MRI of the breast may be indicated after discussion at a breast MDT. FDG PET-CT is only indicated in patients with cervical lymphadenopathy and a normal ENT panendoscopy, or in other patients after MDT discussion. Biopsy: Biopsy (trucut if possible) should be performed if appropriate. If patients are felt to be unfit, advice should be sought from oncology re: the appropriateness of performing a biopsy. Patients with solitary liver, brain, lung, skin or bone tumours need MDT review before biopsy is attempted because biopsy biopsy may affect the outcome of radical treatment. Immunohistochemistry for adenocarcinoma of unknown origin will include CK7, CK20, TTF-1, PLAP, ER in women, PSA in men. Additional immunohistochemistry will be performed as indicated if not an adenocarcinoma. 5

6 Which Multidisciplinary team meetings (MDT) should these patients be discussed in? Please see Local Information for details of the Network CUP / MUO MDT video conference link with Bristol. See local information for details of how and when to submit CUP / MUO MDT referrals and complete the attached Cancer of Unknown Primary MDT Request Form. Referral to the relevant MDT for advice can be made using the information found in the local information section. Referrals must contain the relevant clinical information and a clear indication of what the MDT is required to review and advise on. Liver tumours and intra-abdominal masses - upper GI MDT, or lower GI MDT if possibly colorectal in origin. Solitary liver lesion - hepatobiliary MDT Malignant ascites / peritoneal disease - as above or gynaecology MDT in women. Lung tumours and pleural effusions - lung MDT. Axillary lymph nodes in women - breast MDT. Men with bone metastases and elevated PSA - urology MDT Squamous cell carcinoma (SCC) of the cervical region, unidentified primary - head and neck MDT. Solitary cerebral metastasis: neuro-oncology MDT. Discuss with dermatology if skin lesions present, especially if in region of lymphadenopathy. Other patients may not fit into clear MDT categories (e.g. multiple brain tumours, inguinal lymph nodes, bone metastases (not thought to be myeloma or prostate cancer), cervical lymph nodes (not squamous cell carcinoma). These should be discussed with oncology / haematology via the AOS. Please see local information for details of how to contact AOS. Apart from the patient groups with a potentially better prognosis, patients with MUO/CUP have a poor prognosis. Therefore, prognostic factors such as performance status, serum albumin, presence of liver or multiple cerebral metastases should be taken into account when deciding whether further investigations or treatment are appropriate. This should be done with the guidance of the oncology and palliative care teams. References: NICE guidance: Diagnosis and management of metastatic disease of unknown primary origin. July 2010. ESMO guidance, 2011. UKONS: Acute Oncology Initial Management Guidelines, 2013 6