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

Differential diagnosis

 small blue round cell tumors

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#