女性生殖系统及盆腔原发性原始神经外胚层肿瘤4例临床病理分析  

Primary primitive neuroectodermal tumor of the female reproductive system and pelvis:a clinicalpathological analysis of four cases

作　　者：罗冬云 何春燕 于海云 王志强 张晶 郑兴征[1] LUO Dong-yun;HE Chun-yan;YU Hai-yun;WANG Zhi-qiang;ZHANG Jing;ZHENG Xing-zheng(Departmentof Pathology,Beijing Obstetrics and Gynecology Hospital,Capital Medical University,Beijing Maternal and Child Health Care Hospital,Beijing 100006,China)

机构地区：[1]首都医科大学附属北京妇产医院/北京妇幼保健院病理科,北京100006

出　　处：《诊断病理学杂志》2024年第6期532-536,共5页Chinese Journal of Diagnostic Pathology

基　　金：北京市级医院课题(FCYY202008)。

摘　　要：目的探讨女性生殖系统原发性原始神经外胚层肿瘤(PNET)的临床病理学特征。方法收集首都医科大学附属北京妇产医院确诊的4例PNET,回顾性分析其临床病理资料,并复习相关文献。结果患者年龄15~63岁,其中发生于卵巢2例,子宫1例,盆腔l例。大体观:4例肿瘤大小不等,呈单个巨块状及多发结节状,实性,切面灰白、灰红色,呈鱼肉样。镜下:肿瘤细胞主要呈巢片状分布,表现为均匀一致的小圆细胞,细胞界限不清,胞质少,核分裂象多见。免疫表型:CD99、Fli-1、Vimentin、Syn均阳性(4/4),CD56(2/3+),NSE(3/3局部+),GFAP(1/2局部+)。随访:术后随访5~25个月,4例均死亡。结论女性生殖系统原发性PNET罕见,病理诊断需结合组织学形态、免疫表型和荧光原位杂交(FISH)检测结果综合分析。目前以手术治疗为主。该肿瘤侵袭性强、易早期发生转移,预后差。Objective To investigate the clinicopathological features of primary primitive neuroectodermal tumor(PNET)of the female reproductive system and pelvis.Methods A retrospective analysis was performed on the clinical and pathological data of four cases with PNET that were diagnosed at our hospital.The relevant literature was reviewed.Results The patients were aged 15-63 years with 2 cases in ovary and 1 case in uterus and pelvis,respectively.In general view,tumors were of variable sizes with a single bulky or multiplenodular appearance and ill-defined.The cut surface of those tumors had asolid,fish-like,white to pinky-grey appearance.Microscopically,tumor consisted of monotonous sheets of small,round cells with little cytoplasm and indistinct cytoplasmic borders,which were distributed in nests.The mitotic figures were common.Immunohistochemical study showed that the tumor cells were positive for CD99(4/4),Fli-1(4/4),Vimentin(4/4),CD56(2/3),NSE(3/3 locally positive)and GFAP(1/2 locally positive).The four patients all died with follow-up by 5-25 months.Conclusion Primary PNET is rare in female reproductive system.The final pathological diagnosis can be made when combined with histological morphology,immunophenotype,and fluorescence in situ hybridization(FISH)detection results.At present,surgery is the main treatment.PNET has a poor prognosis,which is aggressive and apt to early metastasis.

关 键 词：原始神经外胚层肿瘤 女性生殖系统 盆腔 免疫组织化学 诊断 

分 类 号：R373.3[医药卫生—病原生物学]