[Desmoplastic small round cell tumor]

ABSTRACT

Desmoplastic small round cell tumor is a recently recognized and rare clinicopathologic entity with distinctive morphologic and immuno histochemical features .Moreover, specific cytogenetics abnormalities and molecular characteristics have been described and confirmed its identity. It is often located at the abdominal cavity, but can exceptionally be located at the cerebral level or some soft tissue and the bones of the members. We report a case of Desmoplastic small round cell involving gluteal muscle and iliac bone at a 25-year-old girl. Our patient had been treated by chemotherapy based on ifosfamide, doxorubicine, etoposide and cis platine. The evolution was marked by the tumoral progress and the death of the patient 18 months after the discovery of the disease. The second case is a 27-year old man .He had a retro peritoneal desmoplastic small round cell tumor treated by surgery alone. The evolution was marked by metastasis and the patient died 10 months after diagnosis. The prognosis of these tumors remains unclear in spite of the mulitidisciplinary treatment. Hence the necessity of new lines of therapeutic research