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Intra‐abdominal desmoplastic small round cell tumor in childhood: case report and review of the literature Volume 90, issue 12, Décembre 2003

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Authors
Service de chirurgie pédiatrique, centre hospitalier universitaire Fattouma‐Bourguiba, 5000 Monastir, Tunisie Laboratoire d‘anatomie et de cytologie pathologiques, faculté de médecine, 5000 Monastir, Tunisie Laboratoire d‘anatomie et de cytologie pathologiques, faculté de médecine, Sousse, Tunisie Service de pédiatrie, centre hospitalier universitaire Sahloul, Sousse, Tunisie

Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinico‐pathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno‐histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor. ▴