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Clinical perspective on desmoplastic small round-cell tumor
Alberto Gil1, Alberto Gomez Portilla, Erwin A Brun
1Washington Cancer Institute, Washington, DC 20010, USA.
Oncology
|November 24, 2004
Summary
Desmoplastic small round-cell tumor (DSRCT) is a rare cancer. Aggressive treatment did not improve survival, but early intervention with surgery and chemotherapy may offer future hope.
Area of Science:
- Oncology
- Rare Cancers
- Peritoneal Surface Malignancy
Background:
- Desmoplastic small round-cell tumor (DSRCT) is a rare, aggressive malignancy primarily affecting young males.
- Characterized by peritoneal surface involvement and a historically poor prognosis with a mean survival of 29 months.
Purpose of the Study:
- To analyze clinical and pathological data of DSRCT patients treated at a single institution.
- To compare outcomes with existing literature data to inform clinical pathway development.
- To evaluate the efficacy of cytoreductive surgery and intraperitoneal chemotherapy.
Main Methods:
- Retrospective analysis of 7 DSRCT patients treated at Washington Hospital Center.
- Comparison with a comprehensive review of DSRCT cases from medical literature.
- Data collection included clinical presentation, tumor distribution, surgical intervention, and survival outcomes.
Main Results:
- Common symptoms include abdominal pain, increased girth, and palpable mass.
- Cytoreductive surgery plus intraperitoneal chemotherapy showed a mean survival of 32 months, not improving upon historical data.
- Two long-term survivors (55 and 101 months) achieved response with systemic chemotherapy; complete surgical removal did not correlate with survival.
Conclusions:
- Aggressive treatment strategies did not significantly improve overall survival, likely due to advanced disease stage at diagnosis.
- Complete surgical resection alone did not correlate with improved survival.
- A comprehensive approach involving early, complete surgical tumor clearing combined with perioperative systemic and intraperitoneal chemotherapy is proposed for future management.