颅内多灶性生殖细胞肿瘤的临床特点及诊治

Clinical features, diagnosis and treatment of intracranial multifocal germ cell tumors

目的：总结颅内多灶性生殖细胞肿瘤的临床特性,并探索合理的治疗方案。方法回顾性分析27例颅内多灶性生殖细胞肿瘤病人的临床资料,颅内共有病灶69个,以同时位于松果体区和鞍上区最常见。最大肿瘤灶位于松果体区15例,鞍上区11例,额叶1例。单纯血浆β-人绒毛膜促性腺激素（β-HCG）增高7例,单纯甲胎蛋白（AFP）增高1例,β-HCG 和 AFP 同时增高1例。接受放疗24例,其中8例联合化疗；放弃治疗3例。结果确诊后接受治疗的24例中,3例完成放疗,正在化疗；检查显示肿瘤病灶均已消失。余19例随访6个月~6.25年,平均3.75年；死亡1例,复发1例,其余17例无复发征象,均能够正常生活。失访2例。结论颅内多灶性生殖细胞肿瘤主要为生殖细胞瘤,考虑系种植转移所致,如能强化治疗,包括全脊髓放疗,预后多良好。

Objective To summarize the clinical features of intracranial multifocal germ cell tumors and explore the reasonable therapeutic strategies. Methods Clinical data of 27 patients with intracranial multifocal germ cell tumor were analyzed retrospectively. There were 69 intracranial lesions in total, which was located mainly in the pineal region and sellar region. The largest lesion was located in pineal region in 15 patients, suprasellar region in 11 and frontal lobe in 1. Elevation of serum β-HCG was seen in 7 patients, elevation of AFP in 1 and elevation of both serum β-HCG and AFP in 1. The radiotherapy was performed on 24 patients including 8 undergoing chemotherapy. And 3 patients quit treatment. Results Among the 24 patients who received surgery, 3 patients who had finished radiotherapy were receiving chemotherapy and the examination showed that all the lesions disappeared. Nineteen patients were followed up for 6 months to 6.25 years with a mean period of 3.75 years. Death occurred in 1 patient, recurrence in 1 and no relapse in 17 patients who had normal lives. Two patients were lost to follow-up. Conclusions Intracranial multifocal germ cell tumors are composed predominantly of germinoma, which are considered to be resulted from the dissemination of tumors. Enhanced treatment including the whole CNS radiotherapy can achieve good prognosis.