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Case 25

Updated: Jan 23

A 30-year-old male was found to have large intra-abdominal mass. Tumor positive for keratin.


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What's the diagnosis

  • Ewing sarcoma

  • Neuroendocrine neoplasms

  • Paraganglioma

  • Desmoplastic small round cell tumor

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Answer

This is a case of desmoplastic small round cell tumor. The key pattern here is solid irregular nests in fibrotic stroma/prominent desmoplastic response. IHC for cytokeratin, desmin (dot-like pattern), and WT1 (antibody to C-terminus). Molecular is EWSR1 gene rearrangement or EWSR1-WT1 fusion transcript.


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Credit: Dr. Dong Ren, MD; UC Irvine
Credit: Dr. Dong Ren, MD; UC Irvine

Case credit: UCSD pathology

Author: Wangpan Jackson Shi, MD

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