Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients
✍ Scribed by Fadi Farhat; Stéphane Culine; Catherine Lhommé; Pierre Duvillard; Patrick Soulié; Guy Michel; Marie-Josée Terrier-Lacombe; Christine Théodore; Miroslava Schreinerova; Jean-Pierre Droz
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 350 KB
- Volume
- 77
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
BACKGROUND.
Desmoplastic small round-cell tumor has been identified as a neoplasm with multidirectional immunohistochemical differentiation. The combination of topographic, morphologic, immunohistochemical, and molecular features of this tumor sets it apart as a pathologic entity. The optimal treatment remains to be determined.
METHODS.
Five adult patients were treated according to a uniform first-line chemotherapy program including cisplatin, etoposide, cyclophosphamide, and either doxorubicin or epirubicin. Chemotherapy was delivered after initial surgery in the four patients with intra-abdominal presentation, and at relapse in the fifth patient who had a paratesticular primary tumor.
RESULTS.
All 4 patients with intra-abdominal disease experienced stability lasting from 4 to 9 months. Only one objective persistent complete response was observed; this was in the patient with a paratesticular primary. No salvage treatment was active in the other four patients who died of progressive disease.
CONCLUSIONS.
Our experience points to a certain degree of chemosensitivity for desmoplastic small round-cell tumors. Despite aggressive treatments, survival rates remain disappointing. Other therapeutic modalities are needed to improve these results.