desmoplastic small round cell tumor

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#