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Published on: May 12, 2019
Urogenital involvement by desmoplastic small round-cell tumor
J Furman1, W M Murphy, Z Wajsman
1Department of Pathology and Laboratory Medicine, University of Florida College of Medicine, Gainesville 32610, USA.
The Journal of Urology
|September 25, 1997
Summary
Desmoplastic small round-cell tumor is a rare, aggressive cancer. Urogenital system involvement is significant, with poor prognosis despite chemotherapy.
Area of Science:
- Oncology
- Uropathology
Background:
- Desmoplastic small round-cell tumor (DSRCT) is a rare, highly malignant neoplasm.
- Typically found in the abdominal cavity, DSRCT also affects genitourinary organs.
Purpose of the Study:
- To characterize the clinicopathologic features of DSRCT in the urogenital system.
- To review the clinical presentation and outcomes of genitourinary DSRCT.
Main Methods:
- Review of 109 reported cases of DSRCT.
- Analysis of 25 cases involving urogenital organs, supplemented by 2 additional cases.
Main Results:
- DSRCT exhibits distinct histological, immunohistochemical, and ultrastructural features.
- A characteristic genetic abnormality is suggested in DSRCT.
- Genitourinary DSRCT cases showed poor prognosis despite multidrug chemotherapy.
Conclusions:
- A notable percentage of DSRCT cases involve genitourinary organs.
- Surgical resection is often not feasible for DSRCT.
- Treatment with chemotherapy yields a poor response rate, with limited disease-free survival.
