Soft tissue
Uncertain differentiation
Desmoplastic small round cell tumor

Topic Completed: 1 November 2012

Minor changes: 5 September 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Desmoplastic small round cell tumor [title] soft tissue

Vijay Shankar, M.D.
Page views in 2019: 9,372
Page views in 2020 to date: 4,831
Cite this page: Shankar V. Desmoplastic small round cell tumor. website. Accessed July 7th, 2020.
Definition / general
  • Distinctive neoplastic condition; not strictly a sarcoma
  • Children and young adults, often adolescent boys
  • Large mass in abdomen or pelvis, accompanied by widespread peritoneal tumor implants
  • Other locations include pleura, thorax, scrotum, CNS
Case reports
Gross images

Images hosted on other servers:

Greater omentum


Site unknown


Microscopic (histologic) description
  • Solid nests of round/oval cells surrounded by cellular desmoplastic stroma
  • Also necrosis, cystic degeneration, glandular arrangements, signet ring-like cells, pseudorosette formations, rhabdoid cells, extensive areas of predominantly spindle cell morphology, carcinoid-like differentiation, adenoid cystic-like configuration
Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.

Various images


Images hosted on other servers:

Various images of unknown origin

Abdominal tumor

Lung tumor



Positive stains
  • WT1 (C-19) (also positive in nephroblastomas)
Molecular / cytogenetics description
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