INTRAABDOMINAL DESMOPLASTIC SMALL ROUND-CELL TUMOR IN CHILDREN - A CLINICOPATHOLOGICAL STUDY

Desmoplastic small round cell tumor (RCT) is a relatively newly recogn ized neoplasm. It has a very distinct morphologic and high-grade polyp henotypic expression with a very poor prognosis. It is commonly seen i n adolescent boys. We describe two cases of intra-abdominal desmoplast ic small RCT in young girls (5 and 11 years of age). In both cases, th e exact origin of tumor in the abdomen could not be established. Histo pathologic examination of the biopsy specimens showed distinct desmopl astic stroma and coexpression of various epithelial, neural, and muscl e markers. These two patients were treated primarily by debulking surg ery followed by adjuvant chemotherapy (RCT II protocol). One of the tw o had a stable disease while the other had a progressive disease while on treatment. Thus our findings support the diverse histogenesis of t his tumor and its poor prognosis.