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
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