A case of massive dermatofibrosarcoma protuberans on the right thigh region

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  • 大腿部に生じた巨大なdermatofibrosarcoma protuberansの1例

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<p>Dermatofibrosarcoma protuberans (DFSP) is known as an infiltrative skin tumor of intermediate malignancy, characterized by high rates of local recurrence and a low risk of metastasis. Microscopic findings show the tumor diffusely infiltrates the dermis and subcutis. The main portion of the tumor is composed of fibroblasts arranged in a storiform pattern, with little nuclear pleomorphism. In deep regions, the tumor intricately indigitates with subcutaneous fat, creating a honeycomb-like appearance. Immunohistochemically, DFSP is characterized by the presence of CD34 in a significant proportion of its cells. There are some histological variants in DFSP, and it is difficult to diagnose subtypes of DFSP, except for typical DFSP. In this report, we describe a case of pedunculated and massive DFSP on the right thigh region of a 68-year-old Japanese man, which showed the histological features of a myxoid type. A definite diagnosis of the case was established using detection of the COL1A1-PDGFB fusion gene. Therefore, detection of the COL1A1-PDGFB fusion gene is useful for the diagnosis of a rare subtype of DFSP.[Skin Cancer (Japan) 2016 ; 31 : 227-232]</p>

Journal

  • Skin Cancer

    Skin Cancer 31 (3), 227-232, 2017

    The Japanese Skin Cancer Society

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