胚胎性癌或具有胚胎性癌成分的生殖细胞肿瘤临床病理特征

Clinicopathological features of embryonic carcinoma or germ cell tumor with embryonic carcinoma component

目的:探讨胚胎性癌(embryonal carcinoma,EC)或具有EC成分的生殖细胞肿瘤的临床病理特征。方法:收集45例EC或具有EC成分的生殖细胞肿瘤患者的临床病例资料,相关病例进行组织学及免疫组织化学分析,并对部分复发病例进行随访。结果:EC或具有EC成分的生殖细胞肿瘤原发于性腺28例(睾丸27例,卵巢1例),性腺外13例(中枢神经系统10例,纵隔1例,椎体1例,腹膜后1例);以转移为首发的4例(腹股沟淋巴结2例,腹膜后1例,肺1例)。原发单纯性EC12例,具有EC成分的混合型生殖细胞瘤29例,其中EC与1种生殖细胞肿瘤成分的混合18例,2种成分的混合10例,3种成分的混合1例。与EC混合的生殖细胞肿瘤成分比例依次为畸胎瘤75.9%(22/29)、卵黄囊瘤34.5%(10/29)、精原细胞瘤20.7%(6/29),绒毛膜癌10.3%(3/29)。EC或EC成分免疫组织化学主要表达情况为人类婆罗双树样基因-4(spalt-like transcription factor 4,SALL4)(45/45),八聚物结合转录因子 4(octamer-binding transcription factor 4,OCT4)(45/45),CD30 (43/45);Ki-67的平均阳性率约80%。经过随访,7例患者出现淋巴结转移,1例腹腔转移,且转移成分均为EC,其中3例死亡。结论:EC常与其他类型的生殖细胞肿瘤成分混合,最常见的混合成分是畸胎瘤,单一的EC几乎均发生于睾丸。EC与EC成分的生殖细胞肿瘤的确诊主要依靠组织学形态及免疫组织化学。EC恶性程度往往高于其他生殖细胞肿瘤成分,容易发生转移,且预后较差。

Objective: To investigate the clinicopathological features of embryonic carcinoma or germ cell tumors with embryonic carcinoma component. Methods: Clinical data of 45 patients with embryonic carcinoma (EC) or germ cells with EC component were collected. The relevant cases were analyzed by histology and immunohistochemistry, and the prognosis was followed up. Results: Twenty-eight cases of EC or germ cell tumors with EC component originated from gonads (27 cases from testis, 1 case from ovary) and 13 cases from extra-gonads (10 cases from central nervous system, 1 case from mediastinum, 1 case from vertebral body, 1 case from retroperitoneal);4 cases were of metastatic origin (2 cases from inguinal lymph nodes, 1 case from retroperitoneum, 1 case from lung). There were 12 primary cases of pure EC, 29 primary cases of mixed type germ cell tumor with EC component including 18 cases of EC mixed with 1 type of germ cell component, 10 cases with 2 components, and 1 case with 3 components. The proportion of tumor components mixed with EC were 75.9%(22/29) for teratoma, 34.5%(10/29) for yolk sac tumor, 20.7%(6/29) for seminoma, and 10.3%(3/29) for choriocarcinoma, respectively. The immunophenotype of EC or EC component was spalt-like transcription factor 4 (SALL4)(45/45), octamer-binding transcription factor 4 (OCT4)(45/45) and CD30 (43/45), and the average positive rate of Ki-67 was about 80%. After follow-up, 7 patients had lymph node metastasis, 1 patient had abdominal metastases, and the metastatic components were all EC, of which 3 patients died. Conclusion: EC always mixes with other types of germ cell tumors, the most common mixed component is teratoma, pure EC occurs almost in the testis. the diagnosis of EC or germ cell tumor with embryonic carcinoma component mainly relies on histological morphology and immunohistochemical expression. The degree of malignancy of EC is often higher than that of other germ cell tumor components, which is prone to metastasis and has a poor prognosis.