Elliott Robert E, Hsieh Kevin, Hochm Tsivia, Belitskaya-Levy Ilana, Wisoff Jessica, Wisoff Jeffrey H
Department of Neurosurgery, New York University School of Medicine, New York, New York, USA.
J Neurosurg Pediatr. 2010 Jan;5(1):30-48. doi: 10.3171/2009.7.PEDS09215.
Optimal treatment of primary and recurrent craniopharyngiomas remains controversial. Radical resection and limited resection plus radiation therapy yield similar rates of disease control and overall survival. The data are much less clear for recurrent tumors. The authors report their experience with radical resection of both primary and recurrent craniopharyngiomas in children and compare the outcomes between the 2 groups.
A retrospective analysis was performed in 86 children younger than 21 years of age who underwent a total of 103 operations for craniopharyngioma between 1986 and 2008; these were performed by the senior author. The goal was resection with curative intent in all patients. Two patients were lost to follow-up and were excluded from analysis. The mean age at the time of surgery was 9.6 years, and the mean follow-up was 9.0 years.
All 57 children with primary tumors underwent gross-total resection (GTR). A GTR was achieved in significantly fewer children with recurrent tumors (18 [62%] of 29). There were 3 perioperative deaths (3%). Tumor recurred after GTR in 14 (20%) of 71 patients. Overall survival and progression-free survival were significantly better in patients with primary tumors at time of presentation to the authors' institution. There were no significant differences in the neurological, endocrinological, visual, or functional outcomes between patients with primary and those with recurrent tumors. Factors negatively affecting overall survival and progression-free survival include subtotal resection (recurrent tumors only), tumor size >or= 5 cm, or presence of hydrocephalus or a ventriculoperitoneal shunt. Prior radiation therapy and increasing tumor size were both risk factors for incomplete resection at reoperation.
In the hands of surgeons with experience with craniopharyngiomas, the authors believe that radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity. While GTR does not preclude recurrence and is more difficult to achieve in recurrent tumors, especially large and previously irradiated tumors, radical resection is still possible in patients with recurrent craniopharyngiomas with morbidity similar to that of primary tumors.
原发性和复发性颅咽管瘤的最佳治疗方案仍存在争议。根治性切除与有限切除加放射治疗的疾病控制率和总生存率相似。而复发性肿瘤的数据则不太明确。作者报告了他们对儿童原发性和复发性颅咽管瘤进行根治性切除的经验，并比较了两组的治疗结果。
对1986年至2008年间86例21岁以下因颅咽管瘤接受了103次手术的儿童进行回顾性分析；这些手术均由资深作者完成。目标是对所有患者进行根治性切除。2例患者失访，被排除在分析之外。手术时的平均年龄为9.6岁，平均随访时间为9.0年。
所有57例原发性肿瘤患儿均接受了全切除（GTR）。复发性肿瘤患儿实现GTR的明显较少（29例中的18例[62%]）。有3例围手术期死亡（3%）。71例患者中有14例（20%）在GTR后肿瘤复发。在作者所在机构就诊时，原发性肿瘤患者的总生存率和无进展生存率明显更高。原发性肿瘤患者和复发性肿瘤患者在神经、内分泌、视觉或功能结果方面没有显著差异。影响总生存率和无进展生存率的负面因素包括次全切除（仅复发性肿瘤）、肿瘤大小≥5 cm、存在脑积水或脑室腹腔分流。先前的放射治疗和肿瘤增大都是再次手术时切除不完全的危险因素。
作者认为，对于有颅咽管瘤手术经验的外科医生来说，初次就诊时进行根治性切除提供了疾病控制和潜在治愈的最佳机会，且发病率可接受。虽然GTR不能排除复发，且在复发性肿瘤中更难实现，尤其是大型和先前接受过放射治疗的肿瘤，但复发性颅咽管瘤患者仍有可能进行根治性切除，其发病率与原发性肿瘤相似。
