Dermatofibrosarcoma protuberans (DFSP) is typically a slowly-growing cutaneous tumor. Clinically, it appears as a reddish and firm nodule or plaque. The tumor shows a biologic behavior intermediate between dermatofibroma (benign) and malignant fibrous histiocytoma. Frequently, it arises on the proximal extremities and trunk. Most patients are adults, often young patients, but it mey be also observed in children. As its name suggests, DFSP is a tumor protruding above the surface of skin and tends ti recur after simple surgical excision (30-50% of cases. In recurrences, the tumor may assume a multinodular appearance and may develop regional satellite lesions. DFSP has not a high propension to distant diffusion and metastases are observed rarely (7%). Histologically, DFSP shows a proliferation of short spindle cells, arranged in storiform pattern (Figs 1-4), involving the dermis (Fig. 1), the subcutaneous tissue e frequently more deep tissue (Figs 2-3). Neoplastic cells have uniform appearance, show modest nuclear pleomorphism and rare motoses, and result to be positive for CD34 (Fig. 4).

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The other pages

Home Page: Dermatopathology - C. Urso, MD

page 1: Melanoma of the skin

page 2: Histologic diagnosis of cutaneous melanoma

page 3: Melanoma arising in a melanocytic nevus

page 4: Infiltration and pseudoinfiltration

page 5: Sweat gland carcinomas

page 6: The sentinel lymph node in cutaneous melanoma

page 7: Bullous cutaneous diseases

page 8: Spitz nevus

page 9: Prognostic factors in cutaneous melanoma

page 10: The dysplastic nevus

page 11: Dermatofibrosarcoma protuberans

page 12: Melanoma in situ
page 13: Merkel cell carcinoma
page 14: Basal cell carcinoma
page 15: Melanoma in paediatric age

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