[Intra-abdominal desmoplastic small round-cell tumors. An entity among peritoneal carcinomatoses in young adults]

Desmoplastic small round-cell tumors in young adults were first described in 1989. They may be revealed by an abdominal mass or a peritoneal carcinomatosis. The diagnosis and the initial treatment are based on debulking surgery. Chemotherapy using doxorubicin, ifosfamide, etoposide and cisplatin can then be proposed. Despite some initial chemosensitivity, prognosis remains poor.