Fibrohistiocytic Skin Cancers: Dermatofibrosarcoma Protuberans, Atypical Fibroxanthoma, and Undifferentiated Pleomorphic Sarcoma
As a group, atypical fibroxanthoma, undifferentiated pleomorphic sarcoma, and dermatofibrosarcoma protuberans (DFSP) are rare cutaneous neoplasms with the potential for significant local destruction. In DFSP, delayed diagnosis is common as the clinical presentation can appear benign. Tissue stains are key for diagnosis of dermatofibrosarcoma protuberans from the benign histologic mimicker dermatofibroma. Debate exists regarding the relationship between atypical fibroxanthoma and undifferentiated pleomorphic sarcoma. The preferred treatment for fibrohistiocytic tumors is Mohs micrographic surgery. Since close monitoring for recurrence is critical, repair options for these commonly large postsurgical defects must be carefully planned. In all cases, preoperative planning and management of patient expectation is paramount to successful therapy.
KeywordsAtypical fibroxanthoma Undifferentiated pleomorphic sarcoma Malignant fibrous histiocytoma Dermatofibroma Dermatofibrosarcoma protuberans Sarcoma
- 10.Patel KU, Szabo SS, Hernandez VS, Prieto VG, Abruzzo LV, Lazar AJ, López-Terrada D. Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays. Human Pathol. 2008;39(2):184–93.CrossRefGoogle Scholar
- 23.Wingard JR, Carter SL, Walsh JT, Kurtzberg J, Small TN, Gersten ID, et al. Results of a randomized, double-blind trial of fluconazole (FLU) vs voriconazole (VORI) for the prevention of invasive fungal infections (IFI) in 600 allogeneic blood and marrow transplant (BMT) patients (abstract). Blood (ASH Annual Meet Abstract). 2007;110:163.Google Scholar