儿童原发性颅内绒毛膜癌的临床特点、诊断与治疗

Clinical features, diagnosis, and treatment of primary intracranial choriocarcinoma in children

目的探讨儿童原发性颅内绒毛膜癌的临床特点、诊断、治疗方法及预后。方法回顾性分析2002年5月至2015年1月首都医科大学附属北京天坛医院神经外科收治的8例原发性颅内绒毛膜癌患者（7例经病理学检查证实，l例为临床诊断）。8例均为男性，平均年龄11岁。7例病变位于松果体区，1例位于鞍区；肿瘤平均直径为3—5cm。术前检查显示，8例患者血和脑脊液人绒毛膜促性腺激素（HCG）均极度升高。8例患者中，3例术前行诊断性放疗，1例术前行立体定向放疗。7例采用经胼胝体一穹隆间入路切除肿瘤，1例行放、化疗。随访时间为1～60个月，定期复查血HCG水平及头颅MRI。结果3例术前行诊断性放疗的患者中，2例出现肿瘤卒中。1例术前行立体定向放疗后出现肿瘤卒中。7例手术患者中，全切除5例，近全切除2例。至随访时4例存活，3例死亡。1例未行手术治疗的患者至随访时仍存活，肿瘤无复发。结论原发性颅内绒毛膜癌是罕见的儿童颅内恶性肿瘤，血HCG和脑脊液HCG明显升高现象有助于诊断。术前放疗可能增加肿瘤卒中风险，切除肿瘤或化疗显效前不推荐放疗。手术及联合化疗可能能够取得较好疗效。对于不宜手术的病例，可以进行化疗，并可追加放疗。

Objective To investigate the clinical features, diagnosis, treatment methods, and prognosis of primary intracranial choriocarcinoma in children. Methods Eight children with primary intracrauial choriocarcinoma （7 were confirmed by pathological examination, 1 was confirmed by clinical diagnosis） admitted to the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from May 2002 to January 2015 were analyzed retrospectively. All the 8 children were males, and their mean age was 11. The lesions of 7 children were in the pineal region and 1 in the sellar region. The mean diameter of choriocarcinomas was 3 - 5 cm. Preoperative examination revealed that the blood and human chorionic gonadotropin （HCG） in blood and cerebrospinal fluid of the 8 children elevated extremely. Of the 8 children, 3 were performed preoperative diagnostic radiotherapy and 1 was performed preoperative stereotactic radiotherapy. Seven children were performed tumor resection via the corpus callosum- interforniceal approach, and 1 was performed radiotherapy and chemotherapy. The follow-up period ranged from 1 to 60 months. The HCG levels and head MRI were reexamined periodically. Results Of the 3 children undergoing diagnostic radiotherapy, 2 had tumor apoplexy. One child had tumor apoplexy after undergoing stereotactic radiotherapy before procedure. Of the 7 surgical children, the tumors of 5 children were resected totally and 2 were resected subtotally. Four of the 7 children survived and 3 died at the time of follow-up. One child without having surgical treatment was still alive and did not have tumor recurrence at the time of follow-up. Conclusions Primary intracranial choriocarcinoma is a rare pediatric intracranial malignant tumor. The significantly increased HCG and cerebrospinal fluid HCG contribute to the diagnosis. The preoperative radiotherapy may increase the risk of tumor apoplexy. Radiotherapy is not recommended before having the marked effect of the tumor resection or chemotherapy. Operation and combined chemotherapy may achieve better efficacy. For children who are not suitable for operation, chemotherapy can be performed, and additional radiotherapy can be given.