Desmoplastic Small Round Cell Tumor Treatment

Sharvaricmi08 Feb, 2022News

DSRCT (desmoplastic small round cell tumour) is an uncommon yet deadly neoplasm that mostly affects teenage and young males. An earlier study discovered a male-to-female ratio of about 5 to 1 and a mean age of 22 years upon diagnosis. Clusters of poorly differentiated tiny round blue cells are inside an extensive fibrosclerotic stroma, as first described in 1989. The chromosomal rearrangement t(11;22)(p13;q12), which results in the fusion of the Ewing's sarcoma (EWSR1) and Wilms' tumour (WT1) genes, distinguishes these cells from epithelial, mesenchymal, myogenic, and neural markers. DSRCT begins in the abdomen and tends to spread peritoneally, with eventual metastasis to distal lymph nodes, the liver, and the lungs.