Lung Metastasis From Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Vulva: A Rare Case Report

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Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, soft tissue sarcoma arising in the dermis. It accounts for 0.1% of all cancers and 1% of all soft tissue sarcomas,1 with an estimated incidence of 0.8 to 4.2 cases per million persons per year, gradually increasing over time.2,3 Vulva represents an uncommon primary site for this mesenchymal neoplasm, as sarcomas comprise 1.8% to 3% of all vulvar malignant tumors.4 Presence of atypia, increased mitotic rates, necroses as well as distinct immunohistochemical patterns delineate fibrosarcomatous transformation, an intermediate- to high-grade sarcoma that appears in 5% to 15% of cases, with implications concerning tumor prognosis. Current treatment modalities have recently changed with the identification of t(17;22)(q22;q13) reciprocal translocation, present in most cases.5 We herein report the second case of metastatic vulvar fiborsarcomatous DFSP (FS-DFSP) and eventually summarize current literature on this specific topic, focusing on the pivotal role of genetic testing in the correct choice of treatment for patients experiencing metastatic disease.
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