Desmoplastic small round cell tumor with primary ovarian involvement: Casereport and review

Background. Desmoplastic small round cell tumor (DSRCT) is a rare, aggressi ve, malignant neoplasm that has recently been characterized. It has not bee n associated with a primary visceral organ. In women, cases are even more r are and often have some ovarian involvement. Case. An 11-year-old girl presented with abdominal pain, nausea, and vomiti ng. A CT scan revealed a large heterogeneous pelvic mass with cystic compon ents and an 8-cm midabdominal mass. During exploratory laparotomy, the pati ent was found to have a pelvic mass measuring 12.9 cm replacing normal ovar ian tissue. The midabdominal mass was also removed. Pathology, cytology, an d immunohistochemistry confirmed a desmoplastic small round cell tumor. Eve n with aggressive surgical and medical intervention, the patient died 11 mo nths after initial diagnosis. Conclusion. Ne present a rare small cell tumor that is associated with ovar ian involvement. The prognosis in these patients is extremely poor and very few survivals have been reported. (C) 2000 Academic Press.