Dermatofibrosarcoma protuberans: Surgical management of a challenging mesenchymal tumor

Wiesmüller F, Agaimy A, Perrakis A, Arkudas A, Horch RE, Grützmann R, Vassos N (2019)


Publication Type: Journal article

Publication year: 2019

Journal

Book Volume: 17

Article Number: 90

Journal Issue: 1

DOI: 10.1186/s12957-019-1627-3

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.

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How to cite

APA:

Wiesmüller, F., Agaimy, A., Perrakis, A., Arkudas, A., Horch, R.E., Grützmann, R., & Vassos, N. (2019). Dermatofibrosarcoma protuberans: Surgical management of a challenging mesenchymal tumor. World Journal of Surgical Oncology, 17(1). https://dx.doi.org/10.1186/s12957-019-1627-3

MLA:

Wiesmüller, Felix, et al. "Dermatofibrosarcoma protuberans: Surgical management of a challenging mesenchymal tumor." World Journal of Surgical Oncology 17.1 (2019).

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