Desmoplastic small round cell tumor

Definition:

Desmoplastic small round cell tumor (DSRCT) is a rare tumor typically involving peritoneum. The concept of DSRCT as a distinct tumor has been strengthened by association with a specific chromosomal abnormality, t(11;22)(pl3;q12) that involves the EWSR1 and WT1 genes. EWSR1 encodes the EWS protein which is a member of the FET family of RNA-binding proteins, while WT1 encodes a zinc-finger transcription factor. Several transcriptional targets of the EWSR1-WT1 chimera have been identified such as Platelet Derived Growth Factor A (PDGFA), IL2 receptor-beta, Myeloid Leukemia Factor 1 (MLF1) or Insulin-like Growth Factor 1 receptor (IGF1-R). Somatic MET and PIK3CA mutations have also been identified.

References:

[1]. Armelle Dufresne, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma. 2012;2012:714986. [Content Brief]

[2]. Paul J Zhang, et al. Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product. Mod Pathol. 2003 Mar;16(3):229-35. [Content Brief]

[3]. Tiziana Negri, et al. New transcriptional-based insights into the pathogenesis of desmoplastic small round cell tumors (DSRCTs). Oncotarget. 2017 May 16;8(20):32492-32504. [Content Brief]

[4]. W L Gerald, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998 Sep;16(9):3028-36. [Content Brief]

[5]. Yunyun Jiang, et al. Novel secondary somatic mutations in Ewing's sarcoma and desmoplastic small round cell tumors. PLoS One. 2014 Aug 13;9(8):e93676. [Content Brief]

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