Department of Pediatric Oncology and Hematology, University Hospital, 48149 Mu¨nster, Germany.
Eur J Endocrinol. 2011 Jul;165(1):17-24. doi: 10.1530/EJE-11-0158. Epub 2011 Apr 13.
Hypothalamic obesity has major impact on prognosis and quality of life (QoL) in childhood craniopharyngioma.
For this study, 120 patients were prospectively recruited during 2001 and 2007 and evaluated after 3 years of follow-up (KRANIOPHARYNGEOM 2000). Body mass index (BMI) and QoL at diagnosis and 36 months after diagnosis were analysed based on the reference assessment of tumour localisation and post-surgical hypothalamic lesions. Treatment was analysed based on the neurosurgical strategy of 50 participating neurosurgical centres, the centre size based on the patient load.
BMI SDS at diagnosis was similar in patients with or without hypothalamic involvement. Surgical lesions of anterior and posterior hypothalamic areas were associated with higher increase in BMI SDS during 36 months post-diagnosis compared with patients without or only anterior lesion (+1.8 BMISD, P=0.033, +2.1 BMISD; P=0.011), negative impact on QoL in patients with posterior hypothalamic lesions. Surgical strategies varied among the 50 neurosurgical centres (three large-sized, 24 middle-sized and 23 small-sized centres). Patients treated in small-sized centres presented with a higher rate of hypothalamic involvement compared with those treated in the middle- and large-sized centres. Treatment in large-sized centres was less radical, and the rates of complete resection and hypothalamic surgical lesions were lower in large-sized centres than those of the middle- and small-sized centres. However, a multivariable analysis showed that pre-operative hypothalamic involvement was the only independent risk factor for severe obesity (P=0.002).
Radical neurosurgical strategies leading to posterior hypothalamic lesions are not recommended due to the potential to exacerbate hypothalamic obesity and impaired QoL. Treatment should be confined to experienced multidisciplinary teams.
颅咽管瘤患儿存在下丘脑性肥胖,对预后和生活质量(QoL)有重大影响。
本研究前瞻性纳入 2001 年至 2007 年的 120 例患者,并在 3 年随访后(KRANIOPHARYNGEOM 2000)进行评估。根据肿瘤定位和术后下丘脑损伤的参考评估,分析诊断时和诊断后 36 个月的体重指数(BMI)和 QoL。根据 50 家参与神经外科中心的神经外科策略以及基于患者人数的中心规模来分析治疗方法。
有或无下丘脑受累的患者,诊断时的 BMI SDS 相似。与无下丘脑损伤或仅存在前下丘脑损伤的患者相比,前、后下丘脑区域的手术损伤与诊断后 36 个月 BMI SDS 升高更明显(+1.8 BMI SDS,P=0.033;+2.1 BMI SDS,P=0.011),且后下丘脑损伤的患者 QoL 受到负面影响。50 家神经外科中心的神经外科策略存在差异(3 家大型、24 家中型和 23 家小型中心)。与在中、大型中心治疗的患者相比,在小型中心治疗的患者下丘脑受累的发生率更高。大型中心的治疗方案不那么激进,且大型中心完全切除率和下丘脑手术损伤率均低于中、小型中心。然而,多变量分析显示,术前下丘脑受累是发生严重肥胖的唯一独立危险因素(P=0.002)。
导致后下丘脑损伤的激进神经外科策略不推荐使用,因为可能会加剧下丘脑性肥胖和 QoL 受损。治疗应仅限于经验丰富的多学科团队。