Myxoid Dermatofibrosarcoma Protuberans With Giant Cell Fibroblastoma Features in an Adult: Case Report and Review.
Chenxu Shi, Ahmed Lazim, Paul J Zhang
✦ AI-curated · Sources linked
Case in brief
A 32-year-old man presented with a myxoid dermatofibrosarcoma protuberans (DFSP) that exhibited features of giant cell fibroblastoma. The diagnosis was confirmed through immunohistochemistry and fluorescence in situ hybridization (FISH) for PDGFB rearrangement, underscoring the complexity of this rare presentation. The case illustrates the importance of molecular techniques in confirming atypical sarcomas.
What made this case unusual
The coexistence of myxoid DFSP with giant cell fibroblastoma features in an adult is exceptionally rare, presenting significant diagnostic challenges.
Diagnostic / clinical pearl
Clinicians should consider molecular confirmation, such as FISH for PDGFB rearrangement, when diagnosing atypical presentations of dermatofibrosarcoma protuberans.
Why it matters
This case emphasizes the need for awareness of rare DFSP variants and the role of advanced diagnostic techniques in ensuring accurate diagnosis.