颅内原发性胚胎性癌的临床特征及预后影响因素分析

Clinical Characteristics and Prognosis of Primary Intracranial Embryonal Carcinoma

目的:探讨颅内原发性胚胎性癌的临床特征及预后影响因素,为该病的临床诊治提供参考依据。方法:回顾性分析我院经病理证实的6例颅内原发性胚胎性癌患者的临床资料,随访并比较患者的预后情况。结果:本组病例5例男性和1例女性,平均年龄9.8岁;5例病变位于松果体区,1例位于鞍区;5例头痛起病,1例表现为多尿;术前血液学检查提示:4例患者甲胎蛋白明显升高,1例患者β亚单位绒毛膜促性腺激素明显升高;3例患者手术全切,平均生存期32.0月,3例患者手术次全切,平均生存期21.0月;4例患者术后接受规范放化疗,平均生存期32.0月,1例仅行化疗,生存期22.0月,1例术后未行放化疗,生存期9.0月。结论:颅内原发性胚胎性癌是一种罕见的颅内肿瘤,多见于年轻男性,病变多位于松果体区;术前血液学检查有助于诊断;患者预后差,手术切除程度是一重要预后影响因素,术后放化疗或可延长患者的生存期。

Objective:To analyze the clinical features and prognostic factors of intracranial primary embryonal carcinoma(EC),and provide references for the clinical diagnosis and treatment of EC.Methods:The clinical data of 6 cases of EC confirmed by pathology in our hospital were retrospectively analyzed and the prognosis was followed up and compared.Results:Of the 6 patients,5 patients were males and 1 was female,their mean age was 9.8 years old.The 1 esions of 5 patients were in the pineal region and l in the sellar region.Of the 6 patients,5 patients presented with headache and 1 presented with diuresis.Highly elevated alpha fetoprotein and beta subunit human chorionic gonadotropin were recorded in 4 and 1 patients’blood,respectively.The mean overall survival time for EC with total resection was 32.0 months.The mean overall survival time for EC with subtotal resection was 21.0 months.The mean overall survival time of the 4 patients undergoing standardized adjuvant chemoradiotherapy was 32.0 months.The survival time of one patient just undergoing chemotherapy was 22.0 months.The survival time of one patient without adjuvant therapy was 9.0 months.Conclusion:EC is a rare subtype of intracranial germ cell tumors with a tendency to present in younger patients and has a male bias,mostly located in the pineal region and has a poor prognosis.Preoperative hematologic examination is helpful for the diagnosis of EC.The extent of resection maybe is an important prognosis factor.Postoperative chemoradiotherapy might help prolong survival time.