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腹膜后原始神经外胚层肿瘤(PNET):病例报告及文献复习

Retroperitoneal primitive neuroectodermal tumor (PNET): case report and review of literature.

作者信息

Koch A, Frigo S, Lecointre L, Hummel M, Akladios C Y, Bergerat J P, Noël G, Wattiez A

出版信息

Eur J Gynaecol Oncol. 2017;38(2):314-318.

Abstract

Primitive Neuroectodermal tumor belongs to the family of Ewing's tumor and is characterized by at (11;22) (q24;ql2) or at (21;22) (q22;ql2) translocation. Retroperitoneal primitive neuroectodermal tumor (PNET) are rare, usually affect young adults, and are often diagnosed late. There is no specific characteristics for imaging. The diagnosis is made on histological examination of the surgical spec- imen or biopsies. Radiotherapy and chemotherapy complete the treatment. The authors report the case of a 26-year-old patient who only had pelvic discomfort. Diagnostic laparoscopy showed a retroperitoneal and retrovesical mass of five centimeters. The patient benefited from adjuvant chemotherapy and radiotherapy. She is free of disease 30 months after treatment.

摘要

原始神经外胚层肿瘤属于尤因肉瘤家族,其特征为t(11;22)(q24;q12)或t(21;22)(q22;q12)易位。腹膜后原始神经外胚层肿瘤(PNET)罕见,通常发生于年轻人,且常诊断较晚。影像学检查无特异性特征。诊断依靠手术标本或活检的组织学检查。放疗和化疗完善治疗方案。作者报道了一例26岁仅出现盆腔不适的患者。诊断性腹腔镜检查显示一个5厘米的腹膜后和膀胱后肿物。该患者接受了辅助化疗和放疗。治疗30个月后无疾病复发。

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