41例原发鞍上＋松果体区生殖细胞瘤治疗分析及策略探讨

Treatment outcomes of primary suprasellar and pineal germinoma and related therapeutic strategies： an analysis of 41 patients

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、IMRT8例；5例病理诊断者进行化放疗。放疗采用6MVX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37．8～50．0Gy，预防照射区剂量17．8，35．0Gy。Kaplan-Meier法进行生存率计算。结果5年样本数26例。5年0S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.