中枢神经细胞瘤的临床特征

Clinical characteristics of central neurocytoma

目的探讨中枢神经细胞瘤的临床特征，以提高对该肿瘤的认识。方法收集本科室1995年1月至2006年12月73例中枢神经细胞瘤的临床资料并进行回顾性分析。结果发病率约占颅内肿瘤的0．3％。男：女=1．28：1，平均发病年龄31．9岁。临床表现：颅内压增高症状53例，头昏头晕18例，肢体乏力15例，视力改变9例。肿瘤主要来源于透明隔50例。肿瘤误诊率64．4％。手术治疗中，肿瘤全切率80．8％，死亡率8．2％。随访到36例，时间6-105个月，无一例肿瘤复发或再长，术后行放疗19例。结论中枢神经细胞瘤多见于年轻人，男性多见，临床表现主要为颅内压增高的症状。肿瘤大多来源于透明隔。本病误诊率高。手术全切是治疗的最佳选择，但手术死亡率高。全切后无需放疗。肿瘤预后良好。

Objective To investigate clinical characteristics of central neurocytoma so as to diagnose correctly and to manage properly this tumor. Method All cases of central neurocytoma operated on in our hospital between Jan. 1995 and Dec. 2006 were reviewed for age, gender, clinical presentation, features of imaging and neuropathology, treatment options and outcome, which follow-up data were obtained from out-patient clinic or from direct contact with the patients. Results There were 41 males and 32 females. The mean age of presentation was 31.9 years. Central neurocytoma comprised 0. 3% of brain tumors. Intracranial hypertension was the first clinical symptom in 72.6% of the patients, dizziness in 24.7%, weakness of limb（s） in 17.8%, visual disturbance in 12.3%, 68.5% of the tumors originated from septum pellucidum. Central neurocytoma were diagnosed pre-operatively only in 26 of 73 patients. The rate of total resection of the tumors was 80.8%. The death rate related to operation was 8.2%. Clinical follow-up was available for 36 of 73 patients, ranging from 6 to 105 months postoperatively. All patients were alive and well at last follow-up and none of patients presented signs of recurrence or progression of the disease. 19 patients performed radiotherapy after operation as an adjuvant treatment. Conclusions Central neurocytomas are rare brain tumors occurring in young adults with a predilection for male. Symptoms of intracranial hypertension are main clinical presentation of the tumors. The majority of the tumors originate from septum pellucidum. Misdiagnosis is more frequent. Total removal is the best treatment option for the tumors. However, death rate of operation is high. It is no necessary to perform radiotherapy after total resection. Central neurocytomas have a favorable prognosis.