Desmoplastic small round cell tumor with primary ovarian involvement: case report and review.
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.
Slomovitz
B M
BM
Division of Gynecologic Oncology, New York Presbyterian Hospital-Cornell University Medical Center, New York, New York 10021, USA.
Girotra
M
M
Aledo
A
A
Saqi
A
A
Soslow
R A
RA
Spigland
N A
NA
Caputo
T A
TA
D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D018294:Neoplasms, Adnexal and Skin Appendage / Q000175:diagnosis; D010051:Ovarian Neoplasms / Q000175:diagnosis / Q000473:pathology*