Diagnostic Pitfalls of Morphological Mimicry and CD34 Heterogeneity in Recurrent or Fibrosarcomatous Dermatofibrosarcoma Protuberans: A Clinicopathological Analysis of Three Molecularly Confirmed Cases With Literature Review.

J Cutan PatholJul 25, 2026 (epub)
Case ReportPathology (Anatomic & Clinical)DermatologyOncology (Medical)

Qian-Qian Chen, Ye Jiang, Jin-Rui Guo et al.

✦ AI-curated · Sources linked

Case in brief

This clinicopathological analysis examined three molecularly confirmed cases of recurrent or fibrosarcomatous dermatofibrosarcoma protuberans (DFSP) with sarcomatous transformation, alongside seven published cases. The tumors showed varied morphology, including patterns resembling undifferentiated pleomorphic sarcoma and myxofibrosarcoma. CD34 staining ranged from diffuse or partial positivity to complete loss, while all cases had a COL1A1-PDGFB fusion or PDGFB rearrangement. Management included surgery, radiotherapy, and imatinib; follow-up showed substantial risks of local recurrence and distant metastasis. Of two imatinib-treated patients, one responded and one was resistant.

What made this case unusual

The combination of sarcoma-like morphology and complete CD34 loss can obscure the diagnosis of fibrosarcomatous DFSP, despite retention of the characteristic molecular alteration.

Diagnostic / clinical pearl

In an atypical recurrent or fibrosarcomatous tumor, absent CD34 staining does not exclude DFSP; molecular detection of COL1A1-PDGFB fusion or PDGFB rearrangement can confirm the diagnosis.

Source

PubMed · PMID 42502207

Published in J Cutan Pathol. This summary was written by xxcode from the publication's abstract and metadata. It is not peer reviewed and is not a substitute for the original article. For clinical decisions, review the original publication.

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