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desmoplastic small round cell tumor

Wednesday 26 November 2003

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant tumor usually present with widespread abdominal serosal involvement. Isolated cases occur in limbs, head and neck, and brain.

Cytogenetics

- t(11;22)(p13;q12) reciprocal translocation

Immunochemistry

- Epithelial and mesenchymal markers were coexpressed in the tumor cells.
- WT1 expression (#10843285#)

Molecular biology

- EWS/WT1 fusion transcript (#8604806#)

Localization

- pancreatic desmoplastic small round cell tumor
- renal desmoplastic small round cell tumor (#17414105#)

Differential diagnosis

- small blue round cell tumors

Case records

- Case #11753
- Case #13651

References

- Barnoud R, Sabourin JC, Pasquier D, Ranchere D, Bailly C, Terrier-Lacombe MJ, Pasquier B. Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors. Am J Surg Pathol. 2000 Jun;24(6):830-6. PMID: #10843285#

Portfolio

  • Peritoneal desmoplastic small round cell tumor
  • Peritoneal desmoplastic small round cell tumor
  • Peritoneal desmoplastic small round cell tumor
  • Cytokeratin immunostaining
  • Desmin immunostaining
  • Desmin immunostaining
  • EMA immunostaining
  • EWSR1/WT1 fusion gene
  • Ki67 immunostaining
  • NH2-WT1 immunostaining
  • Desmoplastic round cell tumor in epiploon
  • Desmoplastic round cell tumor in epiploon