General
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• Similar to counterpart in skin (see skin-nonmelanocytic tumor chapter)
• ICD-0: 8090/3

Epidemiology
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• Very rare

Sites
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• Most cases arise in skin of shaft, may be multicentric

Etiology
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• No evidence of HPV infection
• Typically develops on sun-exposed skin and is closely associated with ultraviolet radiation

Clinical features
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• Usually ages 40+ years (J Urol 1994;152:1557)
• Slowly growing hyperpigmented tumor
• Risk factors include ultraviolet and ionizing radiation, arsenic ingestion, immunosuppression, and inherited syndromes such as nevoid basal cell carcinoma syndrome and xeroderma pigmentosum
• Extremely low metastatic potential (J Am Acad Dermatol 2001;45:68)

Prognostic factors
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• Multicentric and large tumors may present more aggressive behavior

Case reports
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• 75 year old man with multicentric tumor and skin metastases (Scand J Plast Reconstr Surg Hand Surg 2002;36:180)

Treatment
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• Excision

Clinical images
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Ulcer almost circumscribing penis and causing penis / scrotal edema

Micro description
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• Regular neoplastic nests with evident peripheral palisading
• No areas of central comedonecrosis
• Frequent myxoid stromal changes

Micro images
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Skin - not necessarily penis

Differential diagnosis
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• Basaloid carcinoma: involves glans not shaft, more pleomorphic neoplastic cells, frequent central comedonecrosis and absence of peripheral palisading

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