desmoplastic small round cell tumor

Definition: 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.

Image

 desmoplastic stroma in desmoplastic small round cell tumor (DSRCT)

• https://twitter.com/histiocytosisX/status/761299544680247296

IHC

 CKAE1/AE3+ (dot)
 vimentin+ (dot)
 WT1+ (C-19 clone)

See also

 tumoral stroma

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