DSRCT: a rare tumor, primarily seen in adolescent males, was first described by Gerald (1989). It is in the family of small round blue cell tumors of infancy and childhood. These particular tumors have increased cellularity, small size, and occur in children without any male predominance nor abdominal mass which is typically disseminated at diagnosis, usually with involvement of regional lymph nodes with metastases to the lungs and liver.

On light microscopy the islands of small round blue cell tumors are in a dense, characteristic desmoplastic stroma. This stroma distinguishes DSRCT from other round cell tumors. It is suggested that this stroma is an intrinsic reaction to the tumor cells as it is also present in the metastases.

The DSRCT’s immuno-histochemical profile is polyphenotypic with coexpression of epithelial (keratins, epithelial membrane antigen), mesenchymal (desmin and vimentin) and neural (neuronal specific enolase) markers which further distinguishes it from other small round cell tumors.

DSRCT is an extremely aggressive tumor. The three patients in this study had brief duration of symptoms (weeks) and all had large tumor burdens that caused gastointesinal or genito-urital compression.

In diagnosing DSCRT, a laparotomy for adequate tissue sampling is essential. Fine needle aspiration or biopsy left insufficient tissue for definitive diagnosis. In addition, CAT scan was found to significantly underestimate the extent of disease.

Prognosis: As per Gerald’s study, 15 out of 16 patients died of their tumors from 6 months to four years.