ADULT DESMOPLASTIC SMALL ROUND-CELL TUMOR S - A NEW ENTITY

Adult desmoplastic small round cell tumors: a new entity, Desmoplastic small round cell tumor is a recently recognized clinicopathologic ent ity with distinctive morphologic and immunohistochemical features. Mor eover, specific cytogenetic and molecular characteristics have been de scribed. We report here five new adult cases. The median age was 22 ye ars. The clinical presentation war intra-abdominal in four patients an d para-testicular in one patient. In patients with intra-abdominal dis ease, the initial surgery consisted of suboptimal debulking in three p atients and only biopsies in the fourth. The patient with para-testicu lar tumor underwent an orchiectomy. Chemotherapy including cisplatin, etoposide, cyclophosphamide and doxorubicin or epirubicin (PAVEP/PEVEP regimen) was delivered after initial surgery in patients with intra-a bdominal presentation or at relapse in the patient with para-testicula r tumor. Only one persistent complete response was obtained and no sal vage treatment was active in the other patients who died of progressiv e disease. A review of the literature concerning the clinicopathologic , cytogenetic and molecular features of this new entity is exposed.