240例性腺外生殖细胞肿瘤患者的临床病理特点和免疫组化分析

李金航; 曹晨; 封琳; 王钰锋; 李菲菲; 刘爱军

doi:10.3969/j.issn.2095-5227.2022.09.004

240例性腺外生殖细胞肿瘤患者的临床病理特点和免疫组化分析

Clinicopathological features and immunohistochemical analysis of extragonadal germ cell tumors: A series of 240 cases

摘要

摘要:
背景性腺外生殖细胞肿瘤(extragonadal germ cell tumors，EGCT)较为少见，准确诊断该类肿瘤对患者的治疗和预后有重要意义。
目的探讨性腺外生殖细胞肿瘤的临床病理特征、组织形态学特点和免疫组织化学表型。
方法回顾性分析2005年12月- 2016年12月解放军总医院第一医学中心病理科诊断的240例性腺外生殖细胞肿瘤患者的临床和病理资料。
结果 240例性腺外生殖细胞肿瘤患者中男性108例，女性132例。患者中位年龄26岁，其中＜18岁57例(23.7%)，≥18岁183例(76.3%)。发生部位：纵隔86例(35.8%)，中枢神经系统39例(16.3%)，骶尾部37例(15.4%)，腹部47例(19.6%)，其他部位31例(12.9%)。组织学类型：成熟性畸胎瘤172例(71.7%)，生殖细胞瘤33例(13.7%)，卵黄囊瘤14例(5.8%)，混合性生殖细胞肿瘤12例(5.0%)，不成熟畸胎瘤5例(2.1%)，胚胎性癌3例(1.3%)，绒毛膜癌1例(0.4%)。甲胎蛋白(alpha fetoprotein，AFP)在卵黄囊瘤中阳性率为92.8%(13/14)，胎盘碱性磷酸酶(placental alkaline phosphatase，PLAP)、CD117、OCT-4在生殖细胞瘤中阳性率为96.7%、90.0%、100%(29/30、18/20、6/6)，细胞角蛋白(cytokeratin，CK)在胚胎性癌中呈阳性，人绒毛膜促性腺激素(β-human chorionic gonadotropin，β-HCG)在绒毛膜癌呈阳性。
结论性腺外生殖细胞肿瘤少见，青年发病为主，女性偏多，好发于纵隔；组织学特点、免疫表型特点与性腺内生殖细胞肿瘤相类似；生殖细胞瘤是最常见性腺外恶性生殖细胞肿瘤。

Abstract:
Background Extragonadal germ cell tumors (EGCT) are relatively rare. Accurate diagnosis of tumors based on clinical features, pathological morphology and immunohistochemical characteristics is of great significance for the treatment and prognosis of patients.
Objective To investigate the clinicopathological features, morphological features and immunophenotypes of EGCT.
Methods A retrospective study was performed on clinical pathological data and morphological features of 240 patients with EGCT admitted to the First Medical Center of Chinese PLA General Hospital from December 2005 to December 2016.
Results Of the 240 cases, there were 108 males and 132 females. The median age of the patients was 26 years old. Among them, there were 57 cases with age younger than 18 years old (23.7%), 183 cases were older than 18 years (76.3%). Tumors were located in mediastinum (86 cases, 35.8%), central nervous system (39 cases, 16.3%), sacrococcygeal area (37 cases, 15.4%), abdomen (47 cases, 19.6%), and other sites (31 cases, 12.9%). Histological types included 172 mature teratomas (71.7%) , 33 germ cell tumors (13.7%) , 14 yolk sac tumors (5.8%) , 12 mixed germ cell tumors (5.0%), 5 cases of immature teratoma (2.1%) , 3 cases of embryonic carcinoma (1.3%) and 1 case of choriocarcinoma (0.4%) . The positive rate of AFP in yolk sac tumors was 92.8% (13/14). The positive rates of PLAP, CD117 and OCT-4 in germ cell tumors were 96.7%, 90.0%, 100% (29/30, 18/20, 6/6), respectively. CK was positive in embryonal carcinoma and β-HCG was positive in choriocarcinoma.
Conclusion Extragonadal germ cell tumors are rare and mainly occur in the mediastinum. EGCT are more common in young women. Its histological and immunophenotypic features are similar to those of intragonadal germ cell tumors, and germinoma is the most common extragonadal malignant germ cell tumor.

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