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Dermatofibrosarcoma protuberans: Treatment

Authors
William M Mendenhall, MD
Mark T Scarborough, MD
Franklin P Flowers, MD
Section Editors
Robert Maki, MD, PhD
June K Robinson, MD
Raphael E Pollock, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive cutaneous soft tissue sarcoma. Approximately 85 to 90 percent of DFSPs are low grade, while the remainder contain a high-grade sarcomatous component (which is usually a fibrosarcoma, designated DFSP-FS) and are considered to be intermediate-grade sarcomas [1]. Although they rarely metastasize (fewer than 5 percent of cases), all DFSP variants have a propensity to recur locally.

This topic review will focus on treatment of DFSP. The epidemiology, molecular pathogenesis, histology, clinical presentation, diagnosis, and staging of DFSP are discussed elsewhere, as are issues specific to DFSP arising in the head and neck region. (See "Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging" and "Head and neck sarcomas", section on 'Desmoids and dermatofibrosarcoma protuberans'.)

LOCALIZED DISEASE

The preferred initial treatment for a localized dermatofibrosarcoma protuberans (DFSP) is resection with pathologically negative margins. Tumor size and location dictate the most appropriate surgical procedure. Given that metastases to lymph nodes are extremely rare [2], there is no role for prophylactic regional node dissection. (See "Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging", section on 'Metastatic disease'.)

Importance of resection margins — One of the characteristic features of DFSP is its capacity to invade surrounding tissues in an eccentric fashion to a considerable distance from the central focus of the tumor, with tumor cells invading subcutaneous tissue in the form of irregular tentacle-like projections through the septa and fat lobules. This fact, coupled with the rarity of DFSP and diagnostic delay, often leads to inadequate initial resection. The risk of a local recurrence is as high as 50 percent with simple excision ("conservative" surgical margins), and it is even higher if the margins are positive [1,3-6]. (See "Dermatofibrosarcoma protuberans: Epidemiology, pathogenesis, clinical presentation, diagnosis, and staging", section on 'Histology'.)

The status of the surgical margins is the most important prognostic factor in patients with DFSP:

                 

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Literature review current through: Nov 2016. | This topic last updated: Mon Mar 14 00:00:00 GMT+00:00 2016.
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