Department of Radiation Oncology, Sheba Medical Center, Ramat Gan, Israel.
Int J Radiat Oncol Biol Phys. 2013 Mar 15;85(4):e193-9. doi: 10.1016/j.ijrobp.2012.10.033. Epub 2012 Dec 11.
Ependymoma is less commonly found in the supratentorial brain and has known clinical and molecular features that are unique. Our single-institution series provides valuable information about disease control for supratentorial ependymoma and the complications of supratentorial irradiation in children.
A total of 50 children with newly diagnosed supratentorial ependymoma were treated with adjuvant radiation therapy (RT); conformal methods were used in 36 after 1996. The median age at RT was 6.5 years (range, 1-18.9 years). The entire group was characterized according to sex (girls 27), race (white 43), extent of resection (gross-total 46), and tumor grade (anaplastic 28). The conformal RT group was prospectively evaluated for neurologic, endocrine, and cognitive effects.
With a median follow-up time of 9.1 years from the start of RT for survivors (range, 0.2-23.2 years), the 10-year progression-free and overall survival were 73% + 7% and 76% + 6%, respectively. None of the evaluated factors was prognostic for disease control. Local and distant failures were evenly divided among the 16 patients who experienced progression. Eleven patients died of disease, and 1 of central nervous system necrosis. Seizure disorders were present in 17 patients, and 4 were considered to be clinically disabled. Clinically significant cognitive effects were limited to children with difficult-to-control seizures. The average values for intelligence quotient and academic achievement (reading, spelling, and math) were within the range of normal through 10 years of follow-up. Central hypothyroidism was the most commonly treated endocrinopathy.
RT may be administered with acceptable risks for complications in children with supratentorial ependymoma. These results suggest that outcomes for these children are improving and that complications may be limited by use of focal irradiation methods.
幕上室管膜瘤较为少见，具有独特的临床和分子特征。我们的单机构系列提供了关于幕上室管膜瘤疾病控制和儿童幕上照射并发症的有价值信息。
共有 50 名新诊断为幕上室管膜瘤的儿童接受了辅助放疗（RT）；1996 年后，36 名患者采用了适形方法。RT 时的中位年龄为 6.5 岁（范围，1-18.9 岁）。根据性别（女孩 27 名）、种族（白人 43 名）、切除程度（大体全切除 46 名）和肿瘤分级（间变 28 名）对整个组进行了特征描述。对适形 RT 组进行了神经、内分泌和认知影响的前瞻性评估。
在幸存者从 RT 开始的中位随访时间为 9.1 年（范围，0.2-23.2 年）后，10 年无进展生存率和总生存率分别为 73%+7%和 76%+6%。评估的因素均与疾病控制无关。16 名进展患者中，局部和远处失败的比例相当。11 例患者死于疾病，1 例死于中枢神经系统坏死。17 例患者存在癫痫发作，4 例患者被认为有临床残疾。认知功能有限的影响仅限于难以控制癫痫发作的儿童。通过 10 年的随访，智商和学业成绩（阅读、拼写和数学）的平均水平在正常范围内。中枢性甲状腺功能减退是最常见的治疗内分泌疾病。
对于幕上室管膜瘤患儿，RT 可在可接受的并发症风险下进行。这些结果表明，这些儿童的预后正在改善，并且并发症可能通过使用局灶性照射方法来限制。
