Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor

Felix Wiesmueller,Nikolaos Vassos,Andreas Arkudas,Abbas Agaimy,Aristotelis Perrakis,Raymund E Horch,Robert Grützmann

doi:10.1186/s12957-019-1627-3

Abstract

IntroductionDermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases.Patients and methodsWe present a retrospective study of nineteen patients who were diagnosed with DFSP and operated at our institution in > 10-year period. We examined the clinicopathological parameters with special emphasis on the margin status regarding the clinical outcome and the follow-up.ResultsA total of eight cases underwent re-excision at our institution following primary excision or incisional biopsy performed at a different institution. Seven cases received excision after incisional biopsy at our institution. Four patients developed recurrent disease following primary excision with histological R0 margins at other institutions and received re-excision at our institution. All excisions at our institution resulted in R0 margins with no recurrence recorded at last follow-up (6 to 175; mean 84 months). The mean margin for those who received resection at our institution was 1.67 cm. Negative margins upon primary excision were achieved using a mean margin width of 2.04 cm. Most common tumor localization was the trunk (10 cases).ConclusionAwareness of this rare entity is important for a prompt diagnosis and a proper management of the disease. The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cm is recommended.

Highlights

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases
Fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) is considered to be intermediate-grade neoplasm with a slightly increased risk of distant metastasis compared to ordinary DFSP [2, 9]
We propose to describe the tumor as a ubiquitous soft tissue sarcoma with a tendency to grow in skin that has been exposed to prior trauma

Summary

Introduction

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases. Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade soft tissue sarcoma accounting for approximately 1–2% of sarcoma with an annual incidence rate of 0.8–4.1 cases per million persons per year [1, 2]. This dermal tumor was first described by Taylor [3], clinically was classified by Darier and Ferrand [4] and later named by Hoffman [5]. Congenital cases have been described, DFSP typically arises in the third decade of Approximately 10–15% of all DFSPs shows transition to a spindle cell sarcoma closely similar to adult fibrosarcoma, frequently associated with increased mitotic activity and variable loss of CD34 expression. Because DFSP arises in the dermis and invades radially through preexisting collagen bundles and deeply along connective tissue septae, its extent of invasion is often difficult to clinically appreciate, and determining the appropriate margin width is frequently challenging

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