男性假两性畸形伴隐睾恶性混合型生殖细胞瘤1例临床病理特征及文献复习  

Clinicopathological characterization of one case of pseudohermaphroditism complicat-ed with malignant mixed germ cell tumor of cryptorchidism and review of the literature

作　　者：刘宇平 吴共发 曾宇婷 刘钰君 黄绮亭 赖剑龙 姚雨江 王明甫 Liu Yuping;Wu Gongfa;Zeng Yuting;Liu Yujun;Huang Qiting;Lai Jianlong;Yao Yujiang;Wang Mingfu(Department of Pathology,Zengcheng District People’s Hospital of Guangzhou,Guangzhou 511300;Department of Radiology,The Third People‘s Hospital Of Hubei Province,Wuhan 430033,China)

机构地区：[1]广州市增城区人民医院病理科,广州511300 [2]湖北省第三人民医院放射科,武汉430033

出　　处：《中国组织化学与细胞化学杂志》2020年第4期349-353,348,共6页Chinese Journal of Histochemistry and Cytochemistry

基　　金：广州市科技计划项目(201804010043)。

摘　　要：目的探讨男性假两性畸形伴隐睾恶性混合型生殖细胞瘤的临床病理特征及意义。方法回顾性分析1例男性假两性畸形伴隐睾恶性混合型生殖细胞瘤的临床病理特征及文献复习。结果患者社会性别女性,31岁,因盆腔肿物破裂出血伴休克行紧急剖腹探查手术。送检盆腔肿物,病理巨检见灰褐暗红色部分破裂肿物,大小10cm×9.5cm×6cm,切面灰黄暗红色,质软,明显出血坏死及囊性变。镜下见肿瘤以绒毛膜上皮癌为主,见少许卵黄囊瘤、胚胎性癌、未成熟性畸胎瘤等恶性生殖细胞肿瘤,肿瘤坏死明显。广泛取材在周边见少许残存的幼稚萎缩的曲细精管和Leydig细胞巢。免疫组织化学表型:绒毛膜上皮癌β-HCG(+),卵黄囊瘤AFP(+),胚胎性癌CD30(+)。CT检查发现双肺多发结节考虑转移瘤,染色体核型为46,XY,随访3个月死亡。结论发生于男性假两性畸形患者隐睾的混合型生殖细胞肿瘤较为罕见,恶性程度高,预后较差,广泛取材寻找到正常性腺组织及联合免疫组化对病理诊断及鉴别诊断具有重要价值。Objective To investigate the clinicopathological characteristics of male pseudohermaphroditism complicated with malignant mixed germ cell tumor of cryptorchidism and its significance.Methods The clinicpathologic features of one case of pseudohermaphroditism with cryptorchidism malignant mixed germ cell tumor were retrospectively analyzed and relative literature was reviewed.Results A 31-year-old female underwent the emergency exploratory laparotomy for rupture and hemorrhage of pelvic mass with shock.The pelvic mass was submitted for pathological examination.Gross inspection showed the tumor was grayish brown and dark red with partial rupture,its size was 10cm×9.5cm×6cm,with yellowish dark red section surface,soft texture,obvious hemor-rhage,necrosis and cystic change.Microscopically,the tumors were mainly choriocarcinoma,with a few malignant germ cell tumors such as yolk sac tumor,embryonal carcinoma and immature teratoma,with obvious necrosis.A few residual immature atrophic semi-niferous tubules and Leydig cell nests were found around the tumor.Immunohistochemical phenotypes:chorionic epithelial carcinoma was positive forβ-HCG,yolk sac tumor was positive for AFP,embryonal carcinoma was positive for CD30.The chest CT scan found multiple nodules in lung,suggesting metastases.The karyotype was 46,XY.The patient died after 3 months follow-up since hospital discharge.Conclusion Mixed germ cell tumor of cryptorchidism in male with pseudohermaphroditism is rare,with high maliganancy and poor prognosis.It is of great value for diagnosis and differential diagnosis to find the normal gonadal tissue with extensive sampling and immunohistochemistry.

关 键 词：假两性畸形 隐睾 混合型生殖细胞瘤 

分 类 号：R737.2[医药卫生—肿瘤]