Desmoplastic small round cell tumor
Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 24 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
● Aggressive neoplasm associated with EWS-WT1 translocation; not strictly a sarcoma
● Usually abdomen or pelvis; rare in salivary glands (Eur Arch Otorhinolaryngol 2008;265:S47)
● 22 year old man with parotid gland tumor (Hum Pathol 1999;30:430)
● 24 year old man with submandibular gland tumor (Hum Pathol 2010;41:438)
● 41 year old man with history of diabetes mellitus (Appl Immunohistochem Mol Morphol 2011;19:70)
● Solid nests of round/oval cells surrounded by cellular desmoplastic stroma; also necrosis, cystic degeneration, glandular arrangements, signet ring-like cells, pseudorosette formations
● May have areas without desmoplastic stroma
Small bowel tumor
● Cohesive clusters of small cells with hyperchromatic nuclei and fine granular chromatin, fibromyxoid desmoplastic stroma
Various images-site unspecified
● Cytokeratin, desmin, neuron-specific enolase, WT1 (nuclear staining), EMA, CD56
Molecular / cytogenetics description
● EWS-WT1 fusion transcript
End of Salivary glands > Sarcomas > Desmoplastic small round cell tumor
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