Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor

Language
en
Document Type
Article
Issue Date
2019-09-16
Issue Year
2019
Authors
Wiesmueller, Felix
Agaimy, Abbas
Perrakis, Aristotelis
Arkudas, Andreas
Horch, Raymund E.
Grützmann, Robert
Vassos, Nikolaos
Editor
Abstract

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.

Patients and methods

We 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.

Results

A 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).

Conclusion

Awareness 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.

Journal Title
World Journal of Surgical Oncology
Volume
17
Citation
World Journal of Surgical Oncology 17 (2019). <https://wjso.biomedcentral.com/articles/10.1186/s12957-019-1627-3>
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