Pierre-Kahn A, Brauner R, Renier D, Sainte-Rose C, Gangemi M A, Rappaport R, Hirsch J F
Service de Neurochirurgie, Hôpital des Enfants Malades, Paris.
Arch Fr Pediatr. 1988 Mar;45(3):163-7.
In order to evaluate the results of surgery and radiotherapy upon craniopharyngiomas in children, the authors review their own series of 50 cases treated from 1968 to 1985 and the literature. After subtotal removal, the recurrence-free survival rate, 10 years postoperatively, was 37%. This rate was significantly higher (72%) when the subtotal removal was followed by irradiation with a dose of 50 to 55 grays: however, deafness and severe neuro-psychological and intellectual sequelae were frequent in these patients. After radical excision the rate of recurrence was the lowest, with a 10 year-recurrence-free survival rate of 88%. The postoperative mortality was low in case of pre-chiasmatic craniopharyngiomas, but high in case of retro-chiasmatic ones. Nevertheless, it appears from the recent literature data that impressive surgical improvements are to be expected from new surgical routes, associated with the use of the most recent technologies. The conclusions of this study are: 1. Radical excision is the treatment of choice; 2. If radical excision is not possible, surgery should be followed by irradiation to lower the risk of recurrence; 3. However, in view of the dangers of radiotherapy to the growing brain, it should be delayed as long as possible, particularly in the case of young children, and used only when tumor recurrence has been demonstrated.
为了评估手术和放疗对儿童颅咽管瘤的治疗效果，作者回顾了他们自1968年至1985年治疗的50例病例系列以及相关文献。次全切除术后，术后10年的无复发生存率为37%。当次全切除后接受50至55格雷剂量的放疗时，该比率显著更高（72%）：然而，这些患者中耳聋以及严重的神经心理和智力后遗症很常见。根治性切除术后复发率最低，10年无复发生存率为88%。视交叉前颅咽管瘤术后死亡率低，但视交叉后颅咽管瘤术后死亡率高。尽管如此，从最近的文献数据来看，预计新的手术路径结合最新技术会带来显著的手术改进。本研究的结论是：1. 根治性切除是首选治疗方法；2. 如果无法进行根治性切除，手术后应进行放疗以降低复发风险；3. 然而，鉴于放疗对发育中大脑的危害，应尽可能推迟放疗，尤其是对于幼儿，仅在证实肿瘤复发时使用。
