Agresta G, Campione A, Veiceschi P, Gallo D, Agosti E, Massimi L, Piatelli G, Consales A, Linsler S, Oertel J, Pozzi F, Tanda M L, Castelnuovo P, Locatelli D
Division of Neurosurgery, Department of Biotechnology and Life Sciences, University of Insubria, ASST Sette Laghi, Luigi Borri, 57, 21100, Varese, Italy.
Head and Neck Surgery and Forensic Dissection Research Center, University of Insubria, Luigi Borri, 57, 21100, Varese, Italy.
J Endocrinol Invest. 2023 Jun;46(6):1219-1232. doi: 10.1007/s40618-022-01993-2. Epub 2022 Dec 22.
Craniopharyngiomas (CPGs) are aggressive brain tumors responsible of severe morbidity in children. The best treatment strategies are under debate. Our study evaluates surgical, pituitary, and hypothalamic outcomes of a tailored staged-surgical approach compared to a single-stage radical approach in children with CPGs.
Multicenter retrospective study enrolling 96 children treated for CPGs in the period 2010-2022. The surgical management was selected after a multidisciplinary evaluation. Primary endpoint includes the inter-group comparison of preservation/improvement of hypothalamic-pituitary function, the extent of resection, and progression-free survival (PFS). Secondary endpoints include overall survival (OS), morbidity, and quality of life (QoL).
Gross Total Resection (GTR) was reached in 46.1% of cases in the single-stage surgery group (82 patients, age at surgery 9 ± 4.7 years) and 33.3% after the last operation in the staged surgery group (14 patients age 7.64 ± 4.57 years at first surgery and 9.36 ± 4.7 years at the last surgery). The PFS was significantly higher in patients addressed to staged- compared to single-stage surgery (93.75% vs 70.7% at 5 years, respectively, p = 0.03). The recurrence rate was slightly higher in the single-stage surgery group. No significant differences emerged in the endocrinological, visual, hypothalamic outcome, OS, and QoL comparing the two groups.
In pediatric CPGs' surgical radicality and timing of intervention should be tailored considering both anatomical extension and hypothalamic-pituitary function. In selected patients, a staged approach offers a safer and more effective disease control, preserving psychophysical development.
颅咽管瘤（CPGs）是侵袭性脑肿瘤，可导致儿童严重发病。最佳治疗策略仍存在争议。我们的研究评估了在患有CPGs的儿童中，与单阶段根治性手术方法相比，定制的分期手术方法的手术、垂体和下丘脑结局。
多中心回顾性研究，纳入2010年至2022年期间接受CPGs治疗的96名儿童。经过多学科评估后选择手术管理方法。主要终点包括两组间下丘脑 - 垂体功能的保留/改善、切除范围和无进展生存期（PFS）的比较。次要终点包括总生存期（OS）、发病率和生活质量（QoL）。
单阶段手术组（82例患者，手术年龄9±4.7岁）46.1%的病例实现了全切除（GTR），分期手术组（14例患者，首次手术年龄7.64±4.57岁，最后一次手术年龄9.36±4.7岁）在最后一次手术后为33.3%。与单阶段手术相比，接受分期手术的患者PFS显著更高（5年时分别为93.75%和70.7%，p = 0.03）。单阶段手术组的复发率略高。两组在内分泌、视觉、下丘脑结局、OS和QoL方面没有显著差异。
在小儿CPGs中，应根据解剖范围和下丘脑 - 垂体功能来定制手术的根治性和干预时机。在选定的患者中，分期方法可提供更安全、更有效的疾病控制，同时保留心理生理发育。
