41例原发鞍上＋松果体区生殖细胞瘤治疗分析及策略探讨

摘要

目的：分析颅内原发鞍上＋松果体区生殖细胞瘤患者的治疗结果及治疗策略的探讨。方法1996—2013年放疗鞍上＋松果体区生殖细胞瘤患者41例，男35例、女6例，年龄5～39岁。经病灶活检病理诊断5例，其余为临床诊断。采用常规放疗33例、IMRT 8例；5例病理诊断者进行化放疗。放疗采用6 MV X 线，照射野有全脑室照射＋瘤区补量6例，全脑照射＋瘤区补量16例，全脑脊髓照射＋瘤区补量19例。肿瘤区照射剂量37．8～50.0 Gy，预防照射区剂量17．8～35.0 Gy。 Kaplan－Meier法进行生存率计算。结果5年样本数26例。5年OS 率为95％、RFS 率为85％。复发转移8例，其中脊髓转移4例、脑室播散1例、脑室周边复发3例。22例未进行脊髓照射者脊髓转移4例，19例进行脊髓照射者无脊髓转移。8例IMRT 者及5例化放疗者随访期内均未出现治疗失败。结论颅内生殖细胞瘤放疗效果好，双部位生殖细胞瘤不进行脊髓照射者失败率较高，建议对双部位病灶的患者进行脊髓照射。%Objective To investigate the treatment outcomes of 41 patients with suprasellar and pineal germinoma who are treated in our department in recent 18 years,and to explore related therapeutic strategies.Methods A total of 41 patients with concurrent suprasellar and pineal germinoma who were treated in our department from January 1996 to August 2013 were enrolled.There were 35 male patients and 6 female patients,and the median age was 16 years (range 5-39 years).Five patients had pathologically confirmed germinoma and 36 patients had clinically diagnosed germinoma.Conventional radiotherapy was performed for 33 patients, and intensity-modulated radiotherapy was performed for 8 patients.Combined-modality chemoradiotherapy was performed for the 5 patients with pathologically diagnosed germinoma.The 6 MV X-ray was applied for radiotherapy;6 patients received whole ventricular irradiation and a boost in tumor region, 16 received whole-brain radiotherapy and a boost in tumor region,and 19 received craniospinal irradiation and a boost in tumor region.The median radiation dose for tumor region was 45.0 Gy (37.8-50.0 Gy),and the median dose for prophylactic irradiation was 25.0 Gy (17.8-35.0 Gy).The survival was caculated using Kaplan-Meier method.Results The number of patients followed were 26 at 5-years.The 5-year overall survival rate and relapse-free survival rate were 95% and 85%,respectively.Relapse and metastasis were noted in 8 patients,and 3 of them died.There were 4 patients with spinal cord metastasis,1 patient with ventricular dissemination,and 3 patients with periventricular relapse.Among the 22 patients who did not undergo spinal irradiation,4(18.2%) experienced spinal cord metastasis,and all the other 19 patients who underwent spinal irradiation did not experience spinal cord metastasis.The 8 patients undergoing intensity-modulated radiotherapy and the 5 patients undergoing combined-modality chemoradiotherapy did not experience treatment failure during follow-up.Conclusions Radiotherapy for intracranial germinoma has a good therapeutic effect,and the patients with suprasellar and pineal germinoma who do not undergo spinal cord irradiation have a high failure rate.It is suggested to perform spinal cord irradiation for patients with suprasellar and pineal lesions.