14例原发性中枢神经系统生殖细胞肿瘤临床病理特征分析

Clinicopathologic Features of 14 Primary Germ Cell Tumors from Central Nervous System

【目的】探讨原发性中枢神经系统(CNS)生殖细胞肿瘤(GCTs)的临床特征。【方法】回顾性分析2018年5月至2020年6月在佛山市第一人民医院诊治的14例中枢神经系统GCTs,分析其临床特征。【结果】发生在鞍区的GCTs有6例,其中4例为生殖细胞瘤,1例是卵黄囊瘤,1例为成熟性畸胎瘤;发生在松果体的2例,其中1例为生殖细胞肿瘤,1例为混合生殖细胞瘤伴胚胎型癌成分;发生在第三脑室1例,为生殖细胞瘤;发生在骶尾部的3例,2例为卵黄囊瘤伴成熟性畸胎瘤,1例为卵黄囊瘤,发生在额叶2例;肿瘤主要以颅内高压及局部压迫症状为主;PLAP和CD117在几乎所有生殖细胞肿瘤中都有表达,AFP在卵黄嚢瘤中表达,胚胎型癌中CD30和CK的表达具有特征性。【结论】原发性中枢神经系统GCTs是一类少见的肿瘤,鞍区好发,根据其生物学特征,选用标志性的免疫组织化学指标进行准确的病理学诊断对临床治疗具有重要的指导意义。

【Objective】To investigate the clinical characteristics of primary central nervous system(CNS)germline cell tumor(GCTs)and to investigate the diagnostic significance of immunohistochemical indexes(CD117,PLAP,CD30,CK and AFP).【Methods】A retrospective analysis of 14 cases of central nervous system GCTs in Foshan First People's Hospital from May 2018 to June 2020 were studied.Clinical characteristics and immunohistochemical results were analyzed.【Results】There were 6 cases of GCTs in sellar region,of which 3 cases were germ cell tumors,1 case was yolk sac tumor and I case was mature teratoma.Two cases occurred in the pineal gland,of which one was a germ cell tumor and the other was a mixed germ cell tumor with embryonic carcinoma component.One patient was a germ cell tumor in the third ventricle.Three cases occurred in the sacrococcyx,of which 2 cases were yolk sac tumor with mature teratoma and 1 case was yolk sac tumor.The last 2 cases occurred in the frontal lobe.The tumors were mainly intracranial hypertension and local compression symptoms.PLAP and CD117 were expressed in almost all germ cell tumors,AFP was expressed in yolk sac tumors,and CD30 and CK were characteristic in embryonic carcinoma.【Conclusion】Primary germ cell tumor of central nervous system is a rare type of tumors,which are prone to occur in sella region.According to the biological characteristics,the selection of landmark immunohistochemical indexes for accurate pathological diagnosis has important guiding significance for clinical treatment.