颅内原发生殖细胞瘤的临床特征

Clinical Characteristics of Primary Intracranial Germinoma

目的探讨颅内原发生殖细胞瘤的临床特征。方法收集华西医院脑外科2000年1月-2009年3月119例颅内原发生殖细胞瘤的临床资料进行回顾性分析。结果发病率约占同期颅内肿瘤的1.1%。男∶女=1.64∶1,平均发病年龄15.4岁。临床表现主要为颅内高压症状及视力视野改变,分别为61、56例;肿瘤主要来源于松果体区及鞍区;64例患者行放疗或放疗加化疗,随访45例。55例患者行手术治疗(其中术后行单纯放疗19例,放疗加化疗5例),随访36例。随访时间3～94个月,平均29.7个月。行放疗和放化疗的患者3、5年生存率明显高于单纯行手术治疗的患者。结论颅内原发生殖细胞瘤多见于儿童及青少年,男性多见,肿瘤大多位于松果体区及鞍区。临床表现位于松果体区者主要为颅内高压症状等,位于鞍区者主要为视力视野改变、尿崩等。放疗联合化疗是主要治疗手段。

Objective To investigate clinical characteristics of primary intracranial germinoma so as to diagnose correctly and to manage this tumor properly. Methods One hundred and nineteen cases of primary intracranial germinoma treated in this hospital from January 2000 to March 2009 were reviewed for age, gender, clinical presentation, imaging features and neuropathology, treatment options and outcome, with follow-up in out-patient clinic or from direct contact with patients. Results Primary intracranial germinoma comprised 1.1% of all brain tumors in simultaneous period. Out of 119 cases, 74 were males and 45 were females. The mean age of presentation was 15.4 years. Sixty-one patients presented with intracranial hypertension, where as 56 patients presented with visual acuity disorder or visual field defect. Most of the tumors originated from pineal area and saddle area. Sixty-four patients underwent radiotherapy or chemotherapy and radiotherapy and clinical follow-up was available for 45 patients. Fifty-five patients underwent surgical therapy, and after surgery, 19 underwent radiotherapy, 5 underwent chemotherapy and radiothera- py. Clinical follow-up was available for only 36 of 55 patients who had surgery. Clinical follow-up ranged from 3 to 94 months. 3- and 5 years survival rate for patients who had radiotherapy or, chemotherapy and radiotherapy were much higher than those who were only surfically treated. Conclusion Primary intracranial germinoma is rare brain tumor occurring in children and adolescences with a predilection for male. Symptom of intracranial hypertension is main clinical presentation in the tumors of pineal area and visual acuity disorder or visual field defect and diabetes insipidus in those of saddle area. The majority of the tumors originate from pineal area and saddle area. Chemotherapy and radiotherapy is the main treatment option for the tumors.