女性生殖道原发原始神经外胚层肿瘤临床病理分析  

Primitive neuroectodermal tumor in female genital tract： a clinicopathologic study

作　　者：唐霄[1] 何英[1] 杨帆[1] 李雷[1] 王洪[1] 姚先莹[1] 杨开选[1] 王平 王琪琳[1] 

机构地区：[1]四川大学华西第二医院病理科,成都610041 [2]妇产科

出　　处：《中华病理学杂志》2012年第11期729-732,共4页Chinese Journal of Pathology

摘　　要：目的探讨女性生殖道原发原始神经外胚层肿瘤（PNET）的临床病理特点。方法收集6例原发于女性生殖道PNET患者的资料，分析其临床病理特点，采用免疫组织化学EnVision法检测相关免疫标志物，并通过荧光原位杂交（FISH）检测EWS基因易位。结果6例患者年龄10—27岁，平均20岁，发生部位分别是外阴和阴道各2例，卵巢和子宫各1例。肿瘤最大径2．0～10．0cm（平均5．4cm），外观呈结节状，切面灰白或粉红色，鱼肉样，质嫩，均伴有出血、坏死。镜下肿瘤细胞呈小圆形，细胞界限不清，胞质少，核深染，染色质凝聚，可见小核仁；肿瘤细胞呈片状或小叶状排列，其中1例可见Homer—Wright菊形团结构。免疫表型：CD99、FLI一1和CD56均为阳性或弥漫阳性（6／6），5例波形蛋白和神经元特异性烯醇化酶阳性（5／6），4例巢蛋白和突触素阳性（4／6），2例S-100蛋白阳性（2／6），仅1例嗜铬粒素A阳性（1／6）。5例EWS易位基因检测阳性（5／6）。2例获得随访，l例于术后17个月死于肿瘤转移，1例随访7个月仍健在。4例失访。结论PNET原发于女性生殖道罕见，可发生在卵巢、子宫、外阴、阴道。选择恰当的免疫标志物（CD99、FLI．1、波形蛋白、CD56等）及FISH检测EWS易位基因可为诊断提供帮助。Objective To study the clinicopathologic features of primitive neuroectodermal tumor （PNET） in female genital tract. Methods Six cases of PNET arising in female genital tract were retrospectively reviewed. The clinicopathologic features, immunohistochemical findings and EWS gene translocation study results were analyzed. Results The age of patients ranged from 10 to 27 years （ mean - 20 years）. The sites of involvement included ovary （1 case） , uterus （1 case） , vulva （2 cases） and vagina （2 cases）. The greatest diameter of the tumor ranged from 2 to 10 cm （mean =5.4 era）. The tumor had nodular appearance and showed grayish-pink fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Histologically, the tumor was composed of malignant small round cells with indistinct cell borders, hyperchromafic nuclei, dense chromatin, tiny nucleoli and scanty cytoplasm. The tumor cells were arranged in sheets or lobules. Homer-Wright rosettes were identified in 1 case. Immunohistochemical study showed that the tumor cells were positive for CD99, FLI-1 and CD56 （6/6）. Focal expression of vimentin （5/6）, NSE （5/6）, nestin （4/6）, synaptophysin （4/6）, S-100 protein （ 2/6 ） and chromogranin A （ 1/6 ） was also demonstrated. EWS gene translocation was detected in 5 cases studied. Follow-up information was available in 2 patients （7 and 17 months of follow up, respectively）. One of them died of tumor metastasis 17 months after diagnosis. The other patient was still alive. Conclusions PNET arising in female genital tract is rare. It mainly involves ovary, uterus, vulva and vagina. Immunohistoehemical study using a panel of antibodies and fluorescence in-situ hybridization play an important role in definitive diagnosis of this rare malignancy.

关 键 词：神经外胚瘤 原始 生殖器肿瘤 女(雌)性 免疫表型分型 

分 类 号：R737.3[医药卫生—肿瘤]