Lauridant-Philippin G, Ledem N, Lemoine F, Gouerant S, Guillemet C, Michel P, Scotte M, Di Fiore F
Digestive Oncology Unit, Department of Hepato-Gastroenterology, Rouen University Hospital, 1, rue de Germont, 76031 Rouen cedex, France.
Gastroenterol Clin Biol. 2010 Apr-May;34(4-5):321-4. doi: 10.1016/j.gcb.2010.04.005. Epub 2010 Jun 2.
Desmoplastic small round cell tumor (DSRCT) is a very rare but aggressive malignancy. It is usually observed in males during adolescent and early adulthood. The tumor primarily affects the intra-abdominal serosal and is characterized by distinctive histological and immunophenotypic features and by the specific reciprocal translocation EWS-WT1. Prognosis is mainly poor with a mean survival approximately of 2.5 years. However, long-term survivals have been reported using aggressive multimodal therapy based on complete surgical excision, systemic chemotherapy and radiotherapy. The addition of hyperthermic intraperitoneal chemotherapy in the multimodal approach has been reported in very few cases but no effect on survival has been clearly demonstrated. We report a case of a 51-year old adult patient presenting with a DSRCT treated with aggressive therapy based on systemic chemotherapy, complete cytoreductive surgery associated with hyperthermic intraperitoneal chemotherapy, resulting in a long term survival of 4 years.
促纤维增生性小圆细胞瘤（DSRCT）是一种非常罕见但侵袭性很强的恶性肿瘤。它通常在青少年和成年早期的男性中出现。该肿瘤主要累及腹腔内浆膜，具有独特的组织学和免疫表型特征以及特定的EWS-WT1相互易位。预后通常较差，平均生存期约为2.5年。然而，据报道，采用基于完全手术切除、全身化疗和放疗的积极多模式治疗可实现长期生存。在多模式治疗中添加腹腔内热化疗的病例极少，且尚未明确证明其对生存期有影响。我们报告一例51岁成年患者，患有DSRCT，接受了基于全身化疗、与腹腔内热化疗相关的完全细胞减灭术的积极治疗，实现了4年的长期生存。
