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Desmoplastic Small Round Cell Tumors: Results of a Four-drug Chemotherapy Regimen in Five Adult Patients by Fadi Farhat; Stéphane Culine; Catherine Lhommé; Pierre Duvillard; Patrick Soulié; Guy Michel; Marie-Josée Terrier-Lacombe; Christine Théodore; Miroslava Schreinerova; Jean-Pierre Droz is a Medicine article available to read on EtoBox.

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## 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 disappoint

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Author

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; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley (ISSN 0008-543X)

Published

1996

Language

EN

Field

Medicine (Health Sciences)