Soft tissue
Other tumors
Desmoplastic small round cell tumor

Author: Vijay Shankar, M.D.

Revised: 9 July 2018, last major update November 2012

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

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

Cite this page: Shankar, V. Desmoplastic small round cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuedesmoplastic.html. Accessed August 17th, 2018.
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

Pancreas

Site unknown

Stomach

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

Images hosted on PathOut server:

Courtesy of Dr. Mark R. Wick

WT1, courtesy of Dr. Mark R. Wick



Images hosted on other servers:

Various images of unknown origin

Abdominal tumor


Lung tumor

Pancreas

Stomach

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