[Article in French]
1Département de médecine, institut Gustave-Roussy, Villejuif, France.
Desmoplastic small round cell tumor is a recently recognized clinicopathologic entity with distinctive morphologic and immunohistochemical features. Moreover, specific cytogenetic and molecular characteristics have been described. We report here five new adult cases. The median age was 22 years. The clinical presentation was intra-abdominal in four patients and para-testicular in one patient. In patients with intra-abdominal disease, the initial surgery consisted of suboptimal debulking in three patients and only biopsies in the fourth. The patient with para-testicular 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-abdominal presentation or at relapse in the patient with para-testicular tumor. Only one persistent complete response was obtained and no salvage treatment was active in the other patients who died of progressive disease. A review of the literature concerning the clinicopathologic, cytogenetic and molecular features of this new entity is exposed.