Jellouli Mohamed, Mekki Mongi, Krichene Imed, Zakhama Abdelfattah, Belghith Mohsen, Jouini Riadh, Sahnoun Lassaad, Rammeh Soumaya, Harbi Abdelaziz, Nouri Abdellatif
Service de chirurgie pédiatrique, centre hospitalier universitaire Fattouma-Bourguiba, 5000 Monastir, Tunisie.
Bull Cancer. 2003 Dec;90(12):1063-6.
Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological 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.
促纤维增生性小圆细胞肿瘤（DSRCT）是一种新的临床病理实体，于1989年被归入小细胞肿瘤组。这种病理情况带来了病因、诊断、治疗及预后方面的问题。事实上，其病因仍不明，由于鉴别诊断众多且存在解剖病理学多态性，仅通过免疫组织化学和细胞遗传学研究才能确诊。其治疗方法尚未很好地规范，尽管治疗取得了进展，但其预后仍然不容乐观。这项工作的目的是报告一例促纤维增生性小圆细胞肿瘤的个人观察病例，并对文献进行综述，以阐明这种罕见肿瘤的流行病学、临床、辅助检查及治疗方面的情况。
