A clinicopathologic review of a case series of dermatofibrosarcoma protuberans with fibrosarcomatous differentiation

Journal of Cutaneous Medicine and Surgery
Paul KuzelThomas G Salopek

Abstract

Dermatofibrosarcoma protuberans with fibrosarcomatous differentiation (DFSP-FS) is a rare variant of DFSP with a more aggressive clinical course, characterized by higher rates of local recurrence, metastasis, and death. We conducted a clinicopathologic review of all DFSP-FS cases that occurred in Alberta, Canada, from 1997 to 2007. Of the 75 DFSP cases reviewed, 4 demonstrated fibrosarcomatous differentiation. Three patients were female and one was male, and the age range was 25 to 76 years. Three tumors invaded to skeletal muscle, whereas one invaded to subcutaneous tissue only. Although perineural invasion was noted in all four cases, none exhibited lymphovascular space invasion. One local recurrence developed, and two of four tumors metastasized. Metastasis was associated with tumor size, tumor necrosis, grenz zone involvement, ulceration, thickness, and tumor grade. One patient died within 5 years of diagnosis. DFSP-FS represents a more aggressive subtype of DFSP. Several features of DFSP-FS may impart a higher risk of metastasis.

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Citations

Aug 21, 2016·The Surgical Clinics of North America·Jeffrey Reha, Steven C Katz
Mar 25, 2020·Indian Journal of Surgical Oncology·Harish VermaGautam Biswas
Jul 12, 2017·Oncology Letters·Kamil PohlodekZdeněk Kinkor
Aug 10, 2020·Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.]·Changyou JingShengji Yu
Aug 3, 2020·Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.]·Renee L D PrideNahid Y Vidal

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