BACKGROUND: Desmoplastic small round-cell tumor has been identified as a neoplasm with multidirectional 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 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.
BACKGROUND:Desmoplastic small round-cell tumor has been identified as a neoplasm with multidirectional 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 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.
Authors: A Bertuzzi; L Castagna; V Quagliuolo; V Ginanni; S Compasso; M Magagnoli; M Balzarotti; A Nozza; L Siracusano; I Timofeeva; B Sarina; H Soto Parra; A Santoro Journal: Br J Cancer Date: 2003-10-06 Impact factor: 7.640