DESMOPLASTIC SMALL ROUND-CELL TUMORS - RESULTS OF A 4-DRUG CHEMOTHERAPY REGIMEN IN 5 ADULT PATIENTS

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 p rogram including cisplatin, etoposide, cyclophosphamide, and either do xorubicin or epirubicin. Chemotherapy was delivered after initial surg ery in the four patients with intra-abdominal presentation, and at rel apse in the fifth patient who had a paratesticular primary tumor. RESU LTS. All 4 patients with intra-abdominal disease experienced stability lasting from 4 to 9 months. Only one objective persistent complete re sponse was observed; this was in the patient with a paratesticular pri mary. No salvage treatment was active in the other four patients who d ied of progressive disease. CONCLUSIONS. Our experience points to a ce rtain degree of chemosensitivity for desmoplastic small round-cell tum ors. Despite aggressive treatments, survival rates remain disappointin g. Other therapeutic modalities are needed to improve these results. ( C) 1996 American Cancer Society.