DEFINITION AND PATHOGENESIS
- Marjolin ulcer is a carcinoma arising in post-traumatic scars
- "Post-traumatic squamous cell carcinoma" a generic term for tumors arising from scars and sinuses
- A keratinocyte carcinoma
IMPORTANCE
- 2nd most common skin cancer
- Population-based case-fatality rate of ~0.7%
- Early dx can only be made by a high index of suspicion which leads to bx; early bx and tx may improve outcome
- Incidence varies
- Overall incidence 250X in renal transplant pts
CLINICAL FEATURES
- Susceptiblity may be higher in Japan, N India, and China due to cultural practices
- ↑ ESR (average 33mm/hr)
- Time from initial injury to tx avarages 17.8 yrs
- Non-healing ulcer after bx of a "benign lesion"
- Can arise from chronic granulomas, leukoplakia, actinic keratosis, cutaneous horns, keratotic lesions (plantar feet)
- Risk factors
- Solar radiation
- Ionizing radiation
- Phototherapy with psoralens
- Prior SCC
- Smoking
- Chronic inflammation or infection
- Squamous carcinoma of the nail bed has been associated with a subungual lipoma and secondary chronic infection
- Chemical exposure
- Arsenicals
- Polycyclic/polyaromatic hydrocarbons)
- Disease states (eg, xeroderma pigmentosa, Bowen’s disease, leukoplakia, epidermis dysplasia verruciformis, epidermolyis bullosa dystrophica)
- Xeroderma pigmentosa: (AD) defective DNA repair after UV radiation
- Epidermis dysplasia verruciformis: defect in cell-mediated immunity, susceptibility to human papilloma virus
- Human papiloma virus
- Actinic keratosis
- 1% progress to SCC
- 60% of SCC arise in an actinic keratosis
- Organ transplant patients
- 65X ↑ risk due to immunosupression
- Staging
- T Primary tumor
- TX Primary tumor cannot be assessed
- T0 No evidence of primary tumor
- Tis Carcinoma in situ
- T1 Tumor ≤2 cm in greatest dimension with less than two high-risk features
- T2 Tumor ≥2 cm in greatest dimension or a tumor of any size with two or more high-risk features
- T3 Tumor with invasion of maxilla, mandible, orbit, or temporal bone
- T4 Tumor with invasion of skeleton (axial or appendicular) or perineural invasion of skull base
- (High-risk features: perineural invasion; location of ear, nonglabrous lip; depth ≥ 2 mm, Clark levle IV; poor- or un- differentiation
RADIOLOGIC FEATURES
- Preop CT of lungs and liver
- MRI may be more useful than CT for regional LN metastases
GROSS PATHOLOGY
- Varying sizes of sinus tracts and chronic ulcers, which may be infected
HISTOLOGIC FEATURES
- Keratinocytes, polygonal cells with abundant cytoplasm
- Keratin pearls
- Invasion of the dermis through the basement membrane
- Grading according to the WHO criteria
- Grade I: well differentiated
- Grade II: moderately well differentiated
- Grade III: poorly differentiated
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Atypical verruca
- Kerato-acanthoma
- Plantar keratosis
DISEASE COURSE AND TREATMENT
- Biopsy
- Shave or punch bx
- Bx the border between normal skin and tumor to avoid sampling only necrotic tissue
- Excision biopsy in small lesions when tension-free closure possible
- 0.0028-5.9% metastasis in primary lesions (usually only in advanced, deeply invasive lesions)
- 50% over-all metastatic rate in post-traumatic lesions (possibly ? incidence with prophylactic XRT to regional LNs for those lesions at higher risk for metastasis)
- 10% for grade I
- 59% for grade II
- 86% for grade III
- Wide local excision, attempt to get 4-6mm margin and one clear tissue (fascia preferably) plane deep to the tumor
- Chemotx for high grade lesions
- Cryotherapy for superficial, well-differentiated, and well-defined lesions of < 1 cm
- 94% 5-year cure rate
- Electrodessication and curettage for superficial, well-differentiated, and well-defined lesions
- 96% 5-year cure rate
- 100% of pts who develop metastases will develop regional LN metastases
- 5-yr survival with metastatic disease 27-39%
- Regional LN dissection of no benefit in improving survival
- Sentinel node bx has been reported
- Metastases in up to 11.2-16.9% with tumor size > 2 cm the independent risk factor
- Metastatic lesions in <2% of cases due to chronic sun exposure
- XRT for patients who are poor surgical candidates
- 90% 5-year cure rate
SPECIAL CONSIDERATIONS
- CARCINOMA (EPITHELIOMA) CUNICULATUM
- Well-differentiated, bulbous mass with multiple sinuses usually on plantar surface with foul-smelling expelled material
- Also has been reported in locations other than the foot
- "Burrowing" invasion of the dermis
- BOWEN’S DISEASE
- Carcinoma in situ, where the basement membrane remains intact
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