Abstract: 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.

Key words: pineal region, mature teratoma, transcallosal approach, interforniceal approach