Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients

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Abstract
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. Cancer 1996;77:1363‐6.