title: Desmoplastic small round cell tumor with primary ovarian involvement: case report and review.
abstract: BACKGROUND
  Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm that has recently been characterized. It has not been associated with a primary visceral organ. In women, cases are even more rare and often have some ovarian involvement.
  
  
  METHODS
  An 11-year-old girl presented with abdominal pain, nausea, and vomiting. A CT scan revealed a large heterogeneous pelvic mass with cystic components and an 8-cm midabdominal mass. During exploratory laparotomy, the patient was found to have a pelvic mass measuring 12. 9 cm replacing normal ovarian tissue. The midabdominal mass was also removed. Pathology, cytology, and immunohistochemistry confirmed a desmoplastic small round cell tumor. Even with aggressive surgical and medical intervention, the patient died 11 months after initial diagnosis.
  
  
  CONCLUSIONS
  We present a rare small cell tumor that is associated with ovarian involvement. The prognosis in these patients is extremely poor and very few survivals have been reported.
authors.affiliation: Division of Gynecologic Oncology, New York Presbyterian Hospital-Cornell University Medical Center, New York, New York 10021, USA.
authors.forename: B M
authors.initials: BM
authors.lastname: Slomovitz
authors.forename: M
authors.initials: M
authors.lastname: Girotra
authors.forename: A
authors.initials: A
authors.lastname: Aledo
authors.forename: A
authors.initials: A
authors.lastname: Saqi
authors.forename: R A
authors.initials: RA
authors.lastname: Soslow
authors.forename: N A
authors.initials: NA
authors.lastname: Spigland
authors.forename: T A
authors.initials: TA
authors.lastname: Caputo
mesh_terms: D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D018294:Neoplasms, Adnexal and Skin Appendage / Q000175:diagnosis; D010051:Ovarian Neoplasms / Q000175:diagnosis / Q000473:pathology*