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Soft tissue tumors
Desmoplastic small round cell tumor
Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 10 March 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
- 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
=========================================================================Gross / clinical images
- 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
| Abdominal tumor|
| Lung tumor|
| Various images of unknown origin|
Molecular / cytogenetics description
- WT1 (C-19) (also positive in nephroblastomas)
End of Soft tissue tumors > Other tumors > Desmoplastic small round cell tumor
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