Recurrent Dermatofibrosarcoma Protuberance with Fibrosarcomatous Transformation: Report of a Case with a Brief Literature Review

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade soft tissue sarcoma originating from the dermis and subcutaneous tissue. Though it represents only about 1% of all soft tissue sarcomas, its transformation to a fibrosarcoma (DFSP-FS) is exceedingly rare. DFSP generally manifests as a slowly enlarging, nontender mass commonly misdiagnosed as a fibrohistiocytic lesion, including dematofibroma or nodular fascitis. Accurate diagnosis at an early stage is important because fibrosarcomatous (FS) transformation influences therapeutic influences therapeutic decisionsa and prognosis. We report a 21-year-old woman with a 3 cm mass in her right anterior thigh diagnosed as a benign fibrohistiocytic lesion. Despite a benign diagnosis and conservative management, it recurred four years later with features characteristic of DFSP-FS. The histopathological assessment showed significant atypia, intense mitotic activity, and a herringbone pattern, confirming its malignant transformation. CD34 positivity in DFSP cells and negativity in fibrosarcomatous areas on immunohistochemistry (IHC) studies supported the diagnosis. The presented case serves as a reminder to include DFSP in the differntial diagnosis of benign fibrohistiocytic lesons. Our patient was not diagnosed with DFSP initially, contributing to intervention delay and progression to an aggressive phenotype. Recurrence and malignant transformation can be avoided by early diagnosis and complete excision with clear margins.

Keywords:

: Dermatofibrosarcoma Protuberance, Fibrosarcoma, Transformation, Molecular

Authors

  • Zahraa Sari Tishreen University, College of Medicine, Syria
  • Dalia Justo American University of the Caribbean Medical School, USA
  • Leah Magdal American University of the Caribbean Medical School, USA
  • Cecilia Alejandra Clendenes-Salazar Peruvian University of Applied Sciences, LIMA, Peru
  • Raffi Paulus American University of the Caribbean Medical School, USA
  • Amruth Alluri American University of the Caribbean Medical School, USA
  • Mariam Sobh American University of the Caribbean Medical School, USA
  • Sharuk Noor Ali American University of the Caribbean Medical School, USA
  • Isabella Seferoglou American University of the Caribbean Medical School, USA
  • Jessica Jahoda Research Writing & Publication (RWP), LLC, NY, USA
  • Mohamed Aziz Research Writing & Publication (RWP), LLC, NY, USA

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Published

03/27/2025

Issue

Vol. 5 No. 1 (2024):

Section

Articles