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Year : 2004  |  Volume : 70  |  Issue : 5  |  Page : 310--312

Childhood onset dermatofibrosarcoma protuberans

Department of Skin & STD, Government Medical College, Mysore, India

Correspondence Address:
B D Sathyanarayana
Department of Skin - STD, Government Medical College, Mysore - 570001, Karnataka
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Source of Support: None, Conflict of Interest: None

PMID: 17642647

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Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, locally aggressive dermal tumor of disputed histogenesis with a tendency for local recurrence. A 16-year-old female presented with hyperpigmented macules, papules, plaques, erythematous nodules and atrophic, indurated lesions ranging from a few millimeters to a few centimeters. Biopsy revealed thinned out epidermis and a diffuse dermal infiltration by fascicles of spindle cells arranged in storiform pattern. The pseudoseptae formed by the tumor cells interdigitating between the subcutaneous adipocytes resulted in a honeycomb pattern. Histochemical findings are helpful, but the chronicity, size, indolent nature, recurrence after surgery and histopathology are more important to arrive at a diagnosis. Wide and deep surgical excision is the treatment of choice.


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Online since 15th March '04
Published by Wolters Kluwer - Medknow