Fibrohistiocytic Skin Cancers: Dermatofibrosarcoma Protuberans, Atypical Fibroxanthoma, and Undifferentiated Pleomorphic Sarcoma

  • Kavita Mariwalla
  • Allison HanlonEmail author


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.


Atypical fibroxanthoma Undifferentiated pleomorphic sarcoma Malignant fibrous histiocytoma Dermatofibroma Dermatofibrosarcoma protuberans Sarcoma 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Mariwalla DermatologyWest IslipUSA
  2. 2.Department of Medicine, Division of DermatologyVanderbilt University Medical CenterNashvilleUSA

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