儿童松果体区成熟畸胎瘤的诊断与治疗

目的探讨儿童松果体区成熟畸胎瘤的诊断及治疗。方法回顾性研究自1998年2月至2008年8月首都医科大学附属北京天坛医院收治的儿童松果体区成熟畸胎瘤44例,分析其诊断特点及治疗过程。结果诊断正确率70.5%。38例合并脑积水患者术前行脑室-腹腔分流术。显微镜下松果体区成熟畸胎瘤全切除34例,近全切10例,无手术死亡病例。结论松果体区肿瘤种类复杂,但患者性别、Parinaud综合征、影像上不均质表现及CT的钙化表现等特点有助于成熟畸胎瘤的诊断。术前治疗脑积水对保证手术安全有益,但具体治疗方案有争议。手术入路需根据具体情况合理选择。

Diagnosis and Treatment of the Pineal Region Mature Teratoma in Children

Objective To investigate the diagnosis and ideal management of pineal region mature teratomas in children. Methods The clinical data of 44 pediatric patients(all were male) with pineal region mature teratoma, who were treated from Feb, 1998 to Aug, 2008 in Beijing Tiantan Hospital, were retrospectively analyzed for the characteristics of diagnosis and therapeutic approaches. Results The preoperative diagnosis of 70.5% of the cases was consistent with the pathological diagnosis made postoperatively. In 38 cases with hydrocephalus cerebral ventricle to peritoneal(V-P) shunt was performed first. Among the 44 cases, the tumors were totally removed in 34, and were subtotally removed in 10. No patient died of surgery. Conclusion In spite of the complexity of tumors in pineal region, there may be some clues such as male, Parinaud syndrome, heterogeneous imaging, calcification on CT, for the diagnosis of mature teratoma. The definitive diagnosis depends on histopathologic examination. The management of hydrocephalus before removing the tumor is beneficial to the safety of the operation. The patients can be cured by total removal of the tumor, but the optimal approach depends on the size and site of the tumor, as well as the preference of the surgeon.