Exenteration. Introduction. The skin. Epidermal malignancies 8/3/2017. Neglected basal cell carcinoma

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Jeremiah Tao, MD, FACS Director, Oculoplastic and Orbital Surgery Associate Professor, UC Irvine Neglected basal cell carcinoma Exenteration Introduction Chief

Other good reasons to remove a lesion: Irritation Eyelid malposition Cosmesis Jeremiah Tao, MD FACS The skin Eyelid skin is the thinnest skin in the body Made

1 Jeremiah Tao, MD, FACS Director, Oculoplastic and Orbital Surgery Associate Professor, UC Irvine Neglected basal cell carcinoma Exenteration Introduction Chief question with any eyelid lesion: Suspicious or not? Other good reasons to remove a lesion: Irritation Eyelid malposition Cosmesis Jeremiah Tao, MD FACS The skin Eyelid skin is the thinnest skin in the body Made up of 2 layers Epidermis Dermis Epidermal malignancies Most common skin malignancy, basal cell carcinoma, arises from the basal layer (undifferentiated cells) Squamous cell carcinoma arises from more superficial epidermal skin cells these squamous cells produce keratin 1

2 Pigment cells Melanocytes, the pigment producing cells, are found in the basal layer of the epidermis Melanin is the pigment produced in melanosomes within the melanocytes Pigment cell malignancy = melanoma Dermis Deep layer of skin Contains skin appendages or adnexa: hair follicles sebaceous glands sweat glands Dermis The most common adnexal malignancy is sebaceous cell carcinoma Skin cancer Most common cancer in US Aging population Longer lifespan Increased outdoor activities Environmental changes Easier to treat when small in size Outline and objectives Suspicious or not, history & pattern recognition Basal cell, squamous cell, melanoma, & Merkel cell Management Malignant or not? History age & history of significant sun exposure are suggestive Lesion growth or change including bleeding Examination Pattern recognition Clinical intuition 2

Characteristics of malignancy Diagnosis starts with suspicion Treatment rests on complete surgical excision The most common skin malignancy is basal cell carcinoma (BCCa) >90% of malignant lid

3 Characteristics of malignancy Diagnosis starts with suspicion Treatment rests on complete surgical excision The most common skin malignancy is basal cell carcinoma (BCCa) >90% of malignant lid lesions Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Ulceration Malignant cells grow rapidly outgrow blood supply central ulceration Benign lesions usually do not ulcerate Central ulceration in basal cell carcinoma Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Lack of tenderness Despite ulceration, often with bleeding, malignant lesions tend not to be painful Pain develops when the tumor causes secondary problems (e.g. necrosis then eyelid malposition and corneal exposure) 3

Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Induration

Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Irregularity Malignant tumors are made up of cell populations growing at

4 Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Induration Malignancies tend to be firm Benign lesions feel more like the surrounding normal skin Palpate the lesion Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Irregularity Malignant tumors are made up of cell populations growing at different rates Differing growth patterns create irregular margins & asymmetric shapes Benign lesions tend to have smooth borders Irregularity (asymmetry) Contrast with benign milia 4

Contrast with benign syringomas Benign intradermal nevus *

lid margin architecture Telangiectasia Dilated & irregular

5 Contrast with benign syringomas Benign intradermal nevus * Classic patient = young, healthy female Irregularity in BCC Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Telangiectasia Dilated & irregular vessels Pathognomonic for basal cell carcinoma Telangiectasia in basal cell carcinoma 5

Differential: benign papilloma Characteristics of malignancy Ulceration

Loss of lid margin architecture Pearly Nodular basal cell carcinoma Rolled

appearance allegedly from proliferating cells in the basal layer of the

6 Differential: benign papilloma Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture Pearly Nodular basal cell carcinoma Rolled translucent margins that are whitish and shiny in appearance Translucent appearance allegedly from proliferating cells in the basal layer of the epidermis Nodular basal cell carcinoma Pearly and nodular basal cell carcinoma 6

7 Differential: amelanotic nevus (benign) Amelanotic nevus (benign) Differential for nodular: epidermal cyst (benign) Fluid filled nodule = benign hidrocystoma Fluid filled nodule = benign hidrocystoma Characteristics of malignancy Ulceration Lack of tenderness Induration Irregularity Telangiectasia Pearly borders Loss of lid margin architecture 7

tumor outgrowing its blood supply Morpheaform BCC Madarosis & eyelid margin architecture change due to basal cell carcinoma

Recurrence should also raise suspicion Basal cell carcinoma (BCC) The most common skin malignancy 90% of eyelid malignancies

8 Notching & madarosis due to BCC Loss of eyelid margin architecture Lash loss, a.k.a., madarosis Eyelid margin destruction May be due to tumor outgrowing its blood supply Morpheaform BCC Madarosis & eyelid margin architecture change due to basal cell carcinoma Characteristics of malignancy Not all the characteristics of malignancy need to be present for the lesion to be malignant Recurrence should also raise suspicion Basal cell carcinoma (BCC) The most common skin malignancy 90% of eyelid malignancies 20% of eyelid tumors in general Related to ultraviolet or actinic damage More common in fair-skinned individuals (tend to burn rather than tan) Lower lid and medial canthus are most common locations (receive more sun than upper eyelid?) 8

Nodular most common Morpheaform or sclerosing Superficial least common on eyelids Nodular basal cell carcinoma Firm, raised, pearly

9 Basal cell carcinoma Neglected basal cell carcinomas Low risk for metastasis May invade locally Mortality 3% Morbidity & mortality most common with: medial canthal lesions h/o treatment with radiation clinically neglected tumors Basal cell carcinoma subtypes Nodular most common Morpheaform or sclerosing Superficial least common on eyelids Nodular basal cell carcinoma Firm, raised, pearly nodule Telangiectasia common Central ulceration may be present rodent ulcer Nodular basal cell carcinoma Nodular basal cell carcinoma 9

10 Nodular basal cell carcinoma Nodular basal cell carcinoma Nodular BCC histology Lobules of small basophilic cells with high mitotic activity Peripheral palisading common Morpheaform basal cell carcinoma More invasive & worse prognosis Margins and invasiveness difficult to discern Firm lesions Ulceration common May mimic surgical scar Morpheaform BCC Morpheaform BCC 10

11 Morpheaform BCC Morpheaform BCC Morpheaform BCC Morpheaform BCC Morpheaform BCC Morpheaform BCC histology Basophilic, malignant basal cells arranged in cords that radiate peripherally No peripheral palisading Surrounding stroma develops fibrosis 11

Superficial BCC Superficial BCC Relatively rare on the face Slightly elevated,

carcinoma (SCC) Much less common than BCC 5-10% of lid malignancies BCC:SCC ratio

nodule or an indurated plaque Squamous cell carcinoma Hyperkeratosis scaly skin

12 Superficial BCC Superficial BCC Relatively rare on the face Slightly elevated, erythematous, scaling patches Superficial BCC Superficial BCC Squamous cell carcinoma (SCC) Much less common than BCC 5-10% of lid malignancies BCC:SCC ratio 40:1 Arises in sun-damaged skin (de novo or from actinic keratosis) Can appear as nodule or an indurated plaque Squamous cell carcinoma Hyperkeratosis scaly skin falls off easily SCC or its precursor, actinic keratosis (AK) ulceration, telangiectasia, & pearly borders 12

diffuse often a large area of subclinical

Can be clinically indistinguishable from

excision Advanced SCC SCC histology Field

13 Squamous cell carcinoma Usually more aggressive than BCC 0.5% metastatic risk if arising de novo 20-40% if arising in area of previously damaged skin Pay attention to pathology report Invasive High atypia Margins may be diffuse often a large area of subclinical tumor involvement Squamous cell carcinoma Can be clinically indistinguishable from BCC May have increased keratin and debris Squamous cell carcinoma Squamous cell carcinoma Residual tumor after previous excision Advanced SCC SCC histology Field of squamous epithelial cells Invasion past basal lamina Keratinization and intercellular bridges present 13

Spread of SCC Direct extension by narrow cellular strands Perineural invasion Lymphatic spread Hematogenous Baso-squamous cell carcinoma Intermediate histology Behavior and treatment are same

6 months SCC differential: actinic keratosis Premalignant skin lesion may become squamous cell carcinoma Looks/feels like rough patches (hyperkeratosis) May have surrounding erythema Usually

14 Spread of SCC Direct extension by narrow cellular strands Perineural invasion Lymphatic spread Hematogenous Baso-squamous cell carcinoma Intermediate histology Behavior and treatment are same as SCC Keratoacanthoma Low-grade squamous cell carcinoma Characteristic large lesion with central crater filled with keratin May come on suddenly (weeks-months) May resolve spontaneously over 6 months SCC differential: actinic keratosis Premalignant skin lesion may become squamous cell carcinoma Looks/feels like rough patches (hyperkeratosis) May have surrounding erythema Usually multiple Actinic keratosis (benign) SCC differential: seborrheic keratosis (benign) Age & sun-related Color varies from flesh color to tan Appear greasy & stuck-on Treatment: shave excision 14

15 Seborrheic keratosis Seborrheic keratosis Keratoacanthoma Crater on histology Molluscum contagiosum also has crater on histology Molluscum bodies Sebaceous cell carcinoma Rare (1 to 5% of eyelid cancers) Arises within sebaceous glands of the skin (adnexal malignancy) BCC, SCC, and sebaceous cell carcinoma comprise >95% of eyelid malignancies 15

Sebaceous cell carcinoma Sebaceous glands in the periocular region: Meibomian glands

eyebrow follicles Sebaceous cell carcinoma is more common in the periocular area than

characteristic appearance masquerader May present as unilateral blepharoconjunctivitis

suggest sebaceous cell carcinoma More common on upper lid more meibomian glands in the

16 Sebaceous cell carcinoma Sebaceous glands in the periocular region: Meibomian glands glands of Zeis associated with lash follicles in the periocular skin in the caruncle eyebrow follicles Sebaceous cell carcinoma is more common in the periocular area than anywhere else due to so many sebaceous glands Sebaceous cell carcinoma No characteristic appearance masquerader May present as unilateral blepharoconjunctivitis or a chronic/recurrent chalazion Yellowish material within any suspicious lesion should suggest sebaceous cell carcinoma More common on upper lid more meibomian glands in the superior tarsus Sebaceous cell carcinoma Sebaceous cell carcinoma Sebaceous cell carcinoma Sebaceous cell CA 16

17 Sebaceous cell carcinoma Chalazion that won t go away Chalazion Chalazion Chalazion Sebaceous cell carcinoma Usually need a full-thickness specimen to make diagnosis Oil red-o stain on fresh tissue stains sebaceous material Foamy cytoplasm seen in dysplastic sebaceous cells diagnostic 17

Sebaceous cell carcinoma Two unusual growth patterns make complete excision difficult 1. Pagetoid spread superficially over large areas; margins not clinically visible 2.

Exenteration Map biopsies assess for peripheral pagetoid spread Take generous margins on full-thickness eyelid biopsy Frozen sections can be unreliable Jeremiah Tao, MD FACS Sebaceous

18 Sebaceous cell carcinoma Two unusual growth patterns make complete excision difficult 1. Pagetoid spread superficially over large areas; margins not clinically visible 2. Multifocal, noncontiguous tumor origins with skip areas between Sebaceous cell carcinoma Map (blind) biopsy low yield Biopsy any abnormal area Now Later Sebaceous cell carcinoma Exenteration Map biopsies assess for peripheral pagetoid spread Take generous margins on full-thickness eyelid biopsy Frozen sections can be unreliable Jeremiah Tao, MD FACS Sebaceous cell carcinoma Why spend so much time on a rare tumor? it can be lethal Regional lymph node metastasis possible The longer the duration of symptoms before treatment, the poorer the prognosis for survival 18

Diameter >6mm in diameter (large lesion) Melanoma May metastasize Risk of metastasis increases with increasing depth of lesion Sentinel

19 Usually pre auricular from eyelids Melanoma Melanoma Eyelid melanomas are rare (<1%) Clinical features new onset or change in existing lesion Asymmetric shape (cannot fold on itself) Borders irregular (uncontrolled growth) Color change or multiple colors within lesion Diameter >6mm in diameter (large lesion) Melanoma May metastasize Risk of metastasis increases with increasing depth of lesion Sentinel node biopsy is usually indicated (unless just melanoma in-situ, or confined to epidermis) Differential for pigmented lesion: nevus (benign) 19

20 Differential for pigmented lesion: nevus (benign) Lid margin nevus Kissing nevi Differential for pigmented lesion: lentigo (benign) Lentigo senilis Ephelides 20

21 Merkel cell carcinoma Merkel cell carcinoma Neuroendocrine skin tumor Rare but may be increasing Highly malignant, potentially lethal Rapidly growing, nontender, red or violaceous nodule with intact skin Infiltrating pattern of growth Metastatic Merkel cell carcinoma Differential: hemangioma Differential: hemangioma Differential: hemangioma Hemangioma Merkel cell carcinoma 21

Malignant eyelid lesions Final diagnosis rests with pathologist Treatment Gold standard = wide local resection with histologic confirmation (3-5+ mm margins), then reconstruction,

Systemic Melanoma - Immune check point inhibitors targeting programmed cell death (Pembrolizumab and Nivolumab) BCC - Hedgehog inhibitors (Vismodegib) Biopsy Techniques Parallel &

22 Malignant eyelid lesions Final diagnosis rests with pathologist Treatment Gold standard = wide local resection with histologic confirmation (3-5+ mm margins), then reconstruction, sentinel nodes for sebaceous cell, melanoma, invasive SCC Other options/adjuncts: Cryo destruction, chemotherapy Chemo or immune therapy for eyelid carcinoma Local Imiquimod Systemic Melanoma - Immune check point inhibitors targeting programmed cell death (Pembrolizumab and Nivolumab) BCC - Hedgehog inhibitors (Vismodegib) Biopsy Techniques Parallel & vertical cuts through tarsus Pentagonal wedge excision: general rules Primary repair for up to 1/3 lid extent Add procedures when >1/3 defect Dog ear deformity at base/apex of pentagon 22

23 Lazy pentagonal wedge excision For margin lesions, can hide wedge under skin/muscle flaps Upper lid use crease Lower lid subciliary Wedge under lid crease incision Jeremiah Tao, MD, FACS Jeremiah Tao, MD, FACS Jeremiah Tao, MD, FACS 23

Jeremiah Tao, MD, FACS Jeremiah Tao, MD, FACS Dailey R, Chavez M.

Ophthal Plast Reconstr Surg. 2011 Jan/Feb;27(1):48-51.

diagnosis Best place to get specimen is at periphery of lesion to include some

24 Jeremiah Tao, MD, FACS Jeremiah Tao, MD, FACS Dailey R, Chavez M. Upper Eyelid Margin Mass Excision Technique: Supraciliary Approach. Ophthal Plast Reconstr Surg Jan/Feb;27(1): Jeremiah Tao, MD, FACS Jeremiah Tao, MD, FACS Incisional vs. excisional biopsy Dailey R, Chavez M. Upper Eyelid Margin Mass Excision Technique: Supraciliary Approach. Ophthal Plast Reconstr Surg Jan/Feb;27(1): Incisional Biopsy Only a portion of the lesion is removed to allow for pathologic diagnosis Best place to get specimen is at periphery of lesion to include some normal tissue Excisional Biopsy The entire gross lesion is removed Jeremiah Tao, MD, FACS 24

Mohs excision Mohs Excision Mohs surgery is a form of excisional

lesion is removed and narrow margins of surrounding tissues are

removed Pre op Squamous cell carcinoma Mohs defect Mohs defect

Post-op Principals of eyelid reconstruction Reconstitute BOTH

lamella: conjunctiva/retractor band/tarsus Reconstitute canthal

25 Mohs excision Mohs Excision Mohs surgery is a form of excisional biopsy performed by specially-trained dermatologists Entire lesion is removed and narrow margins of surrounding tissues are examined with frozen sections In theory, minimal normal tissue is removed Pre op Squamous cell carcinoma Mohs defect Mohs defect often still require wedge due to remnant tarsus Mohs defect Post-op Principals of eyelid reconstruction Reconstitute BOTH eyelid lamella Anterior lamella: skin/orbicularis oculi Posterior lamella: conjunctiva/retractor band/tarsus Reconstitute canthal attachments Reconstitute lacrimal drainage system Pre op Primary closure Mohs defect 25

26 BCC BCC Mohs Defect Closure with forehead and cheek rotation flaps Mohs' defect Final result Basal cell carcinoma Basal cell carcinoma Pre op Mohs defect Intra op Intra op Mustarde flap Mustarde flap Basal cell carcinoma Pre op Mohs defect 26

27 Post op Lateral rotational flap Post op Mohs defect Lateral rotational flap Post op Basal cell pre-op Intra-op after frozen sections Immediate post op Thank You!! 27

Technicians & Nurses Program

ASCRS ASOA Symposium & Congress Technicians & Nurses Program May 6-10, 2016 New Orleans Evaluation and Treatment of Eyelid Malignancies Richard C. Allen MD PhD FACS Professor Section of Ophthalmology Dept.