Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: a case report

Czech version

Authors: Jan Hrudka ¹; Martin Charvát ²; Petr Grossmann ³; Zdeněk Kinkor ³
Authors‘ workplace: Ústav patologie 3. lékařské fakulty Univerzity Karlovy a Fakultní nemocnice Královské Vinohrady, Praha. ¹; Klinika plastické chirurgie 3. lékařské fakulty Univerzity Karlovy a Fakultní nemocnice Královské Vinohrady, Praha. ²; Šiklův ústav patologie, Fakultní nemocnice Plzeň, Lékařská fakulta Univerzity Karlovy v Plzni a Bioptická laboratoř s. r. o., Plzeň. ³
Published in: Čes.-slov. Patol., 56, 2020, No. 2, p. 89-93
Category: Original Article

Overview

Dermatofibrosarcoma protuberans is a quite rare local aggressive tumor of dermis and subcutis, revealing characteristic morphology and chromosomal translocation (17; 22)(q21;q13) with gene fusion COL1A1-PDGFB. The tumour almost never metastasizes and complete excision signs an excellent prognosis. Approximately in 10% of cases, dermatofibrosarcoma undergoes a fibrosarcomatous transformation associated with metastatic disease and worse prognosis. In this paper, we refer a case of a male patient with subcutaneous tumor in back region, in which the small biopsy lead to diagnosis of a spindle cell sarcoma. However, only the histopathological examination of the entire tumor in the material from the radical surgery detected the dermatofibrosarcoma protuberans with fibrosarcomatous transformation. Both components of the tumor showed the characteristic genetic alteration. Identification of fibrosarcomatous component within the DFSP matters in prognosis. Distinction between fibrosarcoma arising within the dermatofibrosarcoma protuberans and fibrosarcoma arising de novo is of therapeutic consequence: the patients with metastatic or inoperable DFSP with fibrosarcomatous transformation may profit form imatinib treatment.

Keywords:

dermatofibrosarcoma protuberans – Fibrosarcoma – fibrosarcomatous – transformation – change

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