Malignant mesenchymal tumors of the penis are very rare and they have vascular origin. We present a case of a 71-year-old man with a painless nodule of 2.0 cm in diameter located in the penile foreskin. There was no history of urinary or sexually transmitted disease. An excisional biopsy revealed a markedly pleomorphic sarcoma resembling atypical fibroxanthoma (AFX) associated with a squamous cell carcinoma in situ. The patient refused a wide re-excision and was free of disease after 36 months. Because the different therapeutic management and prognosis, differential diagnosis should be made with sarcomatoid squamous cell carcinoma and melanoma: A diagnosis of AFX or malignant fibrous histiocytoma may be considered only after the complete exclusion of these two entities.
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