Ossifying fibromyxoid tumors (OFT) of soft parts are very rare neoplasms recently described by Enzinger et al. (1). They usually present as well-circumscribed, slow-growing, asymptomatic subcutaneous masses. A characteristic finding is a fibrous capsule and an incomplete peripheral shell of mature bone. Although most cases behave as benign tumors, a high rate of local recurrences have been described. We describe a 43-year-old male with a perianal subcutaneous tumor of 10 years evolution. The histopathology was compatible with OFT. Due to the subcutaneous location of OFT, both the dermatologist and dermatopathologist need to know about this rare tumor.
We report a 29-year-old woman who had prominent cutaneous markers of tuberous sclerosis, with subependymal nodules and renal cysts on computerized tomographic scan, who also showed multiple angiokeratomas widely distributed on the buttocks and posterior thighs. Enzymatic studies ruled out Fabry's disease and other lysosomal storage disorders. This is the first reported association of widespread angiokeratomas and tuberous sclerosis.
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