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Dermatofibrosarcoma Protuberans

  • Jennifer Hau
  • Shang I. Brian JiangEmail author
Chapter

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing sarcoma. DSFP has low metastatic potential but may be locally aggressive, exhibiting extensive subclinical extension and a high local recurrence rate. Treatment of disease centers on complete surgical resection with emphasis on negative histologic margins and maintaining good function and cosmesis. Treatment options include Mohs micrographic surgery (MMS), modified MMS, wide local excision (WLE), and WLE with circumferential and peripheral deep-margin pathologic assessment. MMS or modified MMS may allow lower recurrence rates than conventional WLE and may be considered first-line options. WLE with circumferential and peripheral deep-margin pathologic assessment may also enable lower recurrence rate with smaller margins. Postoperative adjuvant radiotherapy may be considered in unresectable disease. Imatinib mesylate may be used for unresectable, recurrent, and/or metastatic cases. Other targeted therapies may be beneficial. Treatment approach should take individual factors into account including patient comorbidities, tumor characteristics, and treatment availability.

Keywords

Dermatofibrosarcoma protuberans Cutaneous sarcoma Mohs micrographic surgery Wide local excision Imatinib mesylate 

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Authors and Affiliations

  1. 1.Private PracticeHoustonUSA
  2. 2.University of California San Diego Health, Department of DermatologySan DiegoUSA

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