子宫原始神经外胚层肿瘤临床病理观察  

Primitive neuroectodermal tumor of the uterus:a clinicopathological analysis

作　　者：王艳茹[1] 周金莲[1] 景青萍[1] 吴继华[1] WANG Yan-ru;ZHOU Jin-lian;JING Qing-ping;WU Ji-hua(Department of Pathology,306 Hospital of PLA,Beijing 100101,China)

机构地区：[1]解放军第306医院病理科,北京100101

出　　处：《诊断病理学杂志》2018年第7期507-510,514,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨子宫原始神经外胚层肿瘤(PNET)的临床表现、病理特点、治疗及预后。方法回顾性分析1例原发于子宫的PNET临床病理资料并进行文献复习。结果患者女性,41岁。主因阴道出血2月余,伴间断性下腹痛10天入院。大体为全子宫弥漫浸润性肿瘤,切面灰黄色、鱼肉状,质软。镜下为小圆细胞恶性肿瘤,伴大片状坏死,部分细胞圆形卵圆形,核空泡状,可见小核仁,部分细胞胞质嗜酸性,核偏位,核分裂象易见。免疫组化:肿瘤细胞:vimentin和CD99(+),Syn局部(+);PAS灶状(+);FISH检测EWSR1基因易位(-)。随访4个多月,肿瘤复发并多发转移。结论子宫PNET是一种罕见的小圆细胞恶性肿瘤,可以分为中枢型和外周型。病理诊断需要结合形态学、免疫组化、特殊染色及分子遗传学,没有标准的治疗方案,以手术、放化疗的综合治疗为主,预后极差。Objective To investigate the clinical manifestations,pathological features,treatment and prognosis of the primitive neuroectodermal tumor（ PNET） of the uterus. Methods The clinical and pathological data of one case of PNET from the uterus were analyzed retrospectively and the literatures were reviewed. Results A female patient was 41 years old,with the main presentation of vaginal bleeding for 2 months and intermittent abdominal pain for 10 days before admission. Grossly,the whole uterus was diffusely infiltrated with a gray-yellow tumor,with fleshy and soft on cut surface.Microscopically,it was a malignant small round cell tumor,with large flake necrosis; some cells were round or oval vesicular nuclei with a small nucleoli,some cells were eosinophilic cytoplasm and nucleus deviation,and numerous mitotic figures were seen. Immunohistochemical staining showed that tumor cells were diffusely positive for vimentin and CD99,with partial expression of Syn; PAS stain was focally positive; EWSR1 gene translocation was not detected by fluorescence in situ hybridization（ FISH）. The patient was followed up for more than 4 months,with the result of recurrence and multiple metastases. Conclusions PNET of the uterus is a rare malignant small round cell tumor,and may be classified into central and peripheral PNET. The pathological diagnosis needs to be combined with morphologic features,immunohistochemistry,special staining and molecular genetics. There is no standard therapy; combined treatment with surgery,radiation and chemotherapy is recommended. The prognosis is poor.

关 键 词：子宫 原始神经外胚层肿瘤 临床病理 

分 类 号：R737.33[医药卫生—肿瘤]