Case report reveals diagnostic challenges of a rare myxoid dermatofibrosarcoma in an adult, highlighting the need for molecular confirmation.
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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Shi et al. (2026) studied this question.
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