Sebaceous carcinoma (SC) is a malignant adenocarcinoma with sebaceous differentiation. The lesion typically arises in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. SC presents as a painless, typically slow-growing, nodular mass and may be multifocal. Histologically, the tumour is characterized by dermal lobules of malignant sebocytic cells with multivesicular and vacuolated clear cytoplasm, surrounded by a fibrovascular stroma. The centre of the tumour cell nests may be necrotic. SCs may be associated with overlying carcinoma in-situ or with extramammary Paget disease, or both, in the surface epithelium and in other epidermal appendages.
Sebaceous carcinoma usually arises in middle-aged to elderly patients (average age 62 years). Women are twice as frequently affected. The risk for local recurrence is 30-40%, the risk for distant metastases 20-25%. Tumour-related mortality is 10-20%
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Highly aggressive extraocular sebaceous carcinoma.
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