132例松果体区肿瘤的临床研究

Clinical study of pineal region tumors： experience with 132 patients

目的 研究松果体区肿瘤的临床表现、影像学特征、肿瘤标记物、病理特征、治疗方法 和临床疗效,探讨其特点和治疗策略.方法 回顾性分析2000年1月至2008年5月间住院治疗的132例松果体区肿瘤患者的资料.结果 132例松果体区肿瘤患者中,男性98例,女性34例,男女之比为2.9：1.临床表现以颅内压升高、眼球运动障碍为主.CT和MRI检查有一定特征,但缺乏特异性的影像表现.132例患者中,合并腩积水117例,占88.6%.甲胎蛋白（AFP）升高14例,人绒毛膜促性腺激素（HCG）升高9例.132例患者中,直接放疗18例,病理活检后放疗7例,手术切除107例.有病理诊断结果 者114例,其中生殖细胞源性肿瘤56例.随访12～132个月,肿瘤复发23例,死亡12例.132例患者的5年生存率为89.3%.结论 松果体区肿瘤常合并脑积水,定性诊断较困难,影像学检查有助于诊断,但特异性较差.结合肿瘤标记物可诊断生殖细胞源性肿瘤.纯生殖细胞瘤可直接选择放化疗,其他类型肿瘤均应开颅行手术治疗.应根据患者的病理诊断结果 ,施行联合放化疗,效果较好.

Objective To review the clinical manifestations, imaging, tumor markers, treatment methods, pathology results and clinical curative effects of pineal region tumors and to evaluate the characteristics and intervention strategies for those tumors. Methods The clinicopathological data of 132 patients with pineal region tumor treated in our department between January 2000 and May 2008 were retrospectively studied.Results A moderate predominance in males was presented. The clinical manifestations of the disease included increased intracranial pressure and ocular movement impairment. There were some features but no regularity and specific appearance on imaging including CT and MRI. 88. 6% of patients associated with hydrocephalus. A high serum level of alpha-fetoprotein （AFP） was presented in 14 cases and high HCG in 9 cases.Eighteen cases received direct radiation therapy and 7 had radiotherapy post biopsy. 107 cases were treated surgically and 63 cases received postoperative adjuvant treatment. 114 cases had pathology results including 56 germ cell tumors. The patients were followed up for 12 ～ 132 months. Recurrence developed in 23 cases and 12 cases died.The 5-year survival rate was 89. 3%. Conclusion Pineal region tumors are often associated with hydrocephalus and this makes preoperative diagnosis difficult. Imaging examination may help diagnosis but less specific. Germ cell tumors may diagnosed by some tumor markers. Radiation therapy is the choice of treatment for pure germinomas.Other types of pineal region tumors should receive surgical treatment. Postoperative adjuvant treatment based on pathology can provide a good prognosis in pineal region tumor.