dermatofibrosarcoma

Dermatofibrosarcoma is a rare tumor, but many of the reported cases arise in children.

They occur mostly in boys and are related to dermatofibrosarcoma protuberans.

Turner died in August 2009 of dermatofibrosarcoma protuberans (a soft tissue cancer).

Early clinical trials indicate that imatinib may be effective in treatment of dermatofibrosarcoma protuberans.

Dermatofibrosarcoma.

Dermatofibrosarcoma protuberans is diagnosed with a biopsy, when a portion of the tumor is removed for examination.

Deep-seated dermatofibrosarcoma protuberans.

Deep penetrating dermatofibroma may be difficult to distinguish, even histologically, from rare malignant fibrohistocytic tumours like dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP)

Imatinib has been reported to be an effective treatment for FIP1L1-PDGFRalpha+ mast cell disease, hypereosinophilic syndrome, and dermatofibrosarcoma protuberans.

Price VE, Fletcher JA, Zielenska M, et al.: Imatinib mesylate: an attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans.

Furthermore, PDGFB translocation to the COL1A1 gene locus has been identified to be responsible for dermatofibrosarcoma protuberans (DFSP).

Other indications for Mohs surgery include dermatofibrosarcoma protuberans, keratoacanthoma, spindle cell tumors, sebaceous carcinomas, microcystic adnexal carcinoma, merkel cell carcinoma, Paget's disease of the breast, atypical fibroxanthoma, leiomyosarcoma, and angiosarcoma.