Giant dermatofibrosarcoma protuberans vulvae: rare clinical presentation and literature review.

Giant dermatofibrosarcoma protuberans vulvae: rare clinical presentation and literature review. J BUON. 2019 May-Jun;24(3):1289-1295 Authors: Jeremic J, Stefanovic A, Jeremic K, Jovic M, Pilic I, Cvetkovic A, Stojanovic M Abstract Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing fibro-cutaneous tumor of low to intermediate grade malignancy. It is characterized by local dermal and subcutaneous infiltration, but also with destructive infiltration of the surrounding tissues (muscle, fascia, and bone). The size of the tumor varies from small nodular to large neglected masses. Males and females are equally affected. The tumor is most often localized in the trunk and the proximal extremities. At a molecular level, more than 90% of all DFSP arise from the translocation of chromosomes 17 and 22. Clinically, it usually occurs in the form of flesh-colored or slightly yellow-brown skin tumor, irregular borders or multinodular appearance. The definitive diagnosis of DFSP is made by biopsy in combination with histological morphology and immunohistochemistry. The standard treatment for DFSP is surgical resection. Radiation treatment is an option for primary inoperable tumors and prior multiple recurrences. There is no consensus about chemotherapy regimens. Imatinib - a tyrosine kinase inhibitor - is approved in Europe for the treatment of inoperable primary tumors, locally inoperable recurrent disease, and metastatic DFSP. The recomm...
Source: Journal of B.U.ON. - Category: Cancer & Oncology Tags: J BUON Source Type: research