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Myxoid Dermatofibrosarcoma Protuberans With Giant Cell Fibroblastoma Features in an Adult: Case Report and Review
Chenxu Shi1, Ahmed Lazim1, Paul J Zhang1
1Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
None:
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous sarcoma with a high local recurrence rate but low metastatic potential. The myxoid subtype of DFSP is exceptionally rare, and its occurrence with giant cell fibroblastoma features, particularly in adults, is even more uncommon, presenting diagnostic challenges. We describe a 32-year-old man with a myxoid DFSP exhibiting giant cell fibroblastoma features. The diagnosis was confirmed by immunohistochemistry and fluorescence in situ hybridization (FISH) for PDGFB rearrangement. This report highlights the diagnostic challenges in this rare entity and emphasizes the importance of molecular confirmation.
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