Mm. Basade et al., INTRAABDOMINAL DESMOPLASTIC SMALL ROUND-CELL TUMOR IN CHILDREN - A CLINICOPATHOLOGICAL STUDY, Pediatric hematology and oncology, 13(1), 1996, pp. 95-99
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.