RESEARCH Desmoplastic small round o e

Full list of author information is available at the end of the articleBackground Desmoplastic small round cell tumour (DSRCT) is a rare but highly aggressive neoplasm that typically occurs in adolescent and young males. An earlier study has found a male-to-female ratio of approximately 5 to 1 and a mean age at diagnosis of 22 years [1]. First described in 1989, it is characterised by clusters of poorly differentiated small round blue cells lying within an abundant fibrosclerotic stroma [2]. These cells co-express epithelial, mesenchymal, myogenic and neural markers [3], but are distinguished by the chromosomal translocation t(11;22)(p13;q12) resulting in the fusion of the Ewing’s sarcoma (EWSR1) and the Wilms ’ tumour (WT1) genes [4]. DSRCT generally develops in the abdomen and have a tendency towards peritoneal spread, with subsequent metastasis to distant lymph nodes, liver and lungs [5]. In this study, we retrospectively reviewed 41 patient