Müller H L, Bueb K, Bartels U, Roth C, Harz K, Graf N, Korinthenberg R, Bettendorf M, Kühl J, Gutjahr P, Sörensen N, Calaminus G
Departments of Pediatrics, University Hospital, Würzburg, Germany.
Klin Padiatr. 2001 Jul-Aug;213(4):244-9. doi: 10.1055/s-2001-16855.
Craniopharyngiomas are tumorous embryogenic malformations. As the survival rate after craniopharyngioma is high (92 %), prognosis and quality of life (QoL) in survivors mainly depend on adverse late effects such as obesity.
We analyzed 214 children and adolescents with craniopharyngioma. The records of 185 patients (86 %) were available for retrospective analysis of weight profiles and risk factors for obesity. Quality of life (QoL) was measured in 145 patients by the Fertigkeitenskala Münster/Heidelberg score (FMH) and in 77 patients by PEDQOL questionnaire.
Eighty-two of 185 patients (44 %) developed severe obesity (body mass index [BMI] > 3 SD). Obese patients were compared with 79 patients (43 %) who kept normal weight (BMI < 2 SD). No differences between obese and normal weight patients were found in terms of gender distribution, age at diagnosis and follow-up period. However, the BMI SDS at the time of diagnosis was higher (p < 0.0001) in patients who developed obesity than in those who did not. Furthermore, obese patients presented with bigger tumors (p < 0.05) and a higher rate of a hydrocephalus requiring a shunt (p < 0.05) and hypothalamic involvement (p < 0.05). The mothers of patients who developed severe obesity had a higher BMI (p < 0.001) at the time of diagnosis. Obese patients had a higher height-SDS at diagnosis (p < 0.05) and at the time of last follow-up (p < 0.05) when compared with normal weight patients. A prediction model for severe obesity after craniopharyngioma was calculated by logistic regression based on the risk factors: patient's BMI > 2 SD at diagnosis (p < 0.05; odds ratio: 16.4), hypothalamic involvement (p < 0.05; odds ratio: 3.4) and maternal BMI > 25 kg/m(2) (p < 0.05; odds ratio: 4.6). Significant increases in BMI (p < 0.001) occurred during the early post-operative period especially during the first three years after diagnosis. FMH percentiles correlated negatively with BMI SDS (Spearman r: - 0.37; p < 0.001). Children with craniopharyngioma rated their QoL more negative (p < 0.05) in regard to physical abilities, cognitive functioning and social functioning when compared with healthy children of the same age group. Severely obese patients with craniopharyngioma estimated their QoL lower (p < 0.05) for all domains except for autonomy, cognition and familial integration in comparison with non-obese patients.
Hypothalamic tumor involvement and familial disposition for obesity are risk factors for the development of severe obesity in patients with craniopharyngioma. As weight gain starts early after diagnosis and severe obesity causes a reduction in QoL, early therapeutic efforts should be considered in patients at risk. To confirm our results the prospective multicenter study Kraniopharyngeom 2000 on children and adolescents with craniopharyngioma was initiated (www.kraniopharyngeom.com).
颅咽管瘤是肿瘤性胚胎发育畸形。由于颅咽管瘤患者的生存率较高（92%），幸存者的预后和生活质量（QoL）主要取决于肥胖等不良晚期效应。
我们分析了214例患有颅咽管瘤的儿童和青少年。185例患者（86%）的记录可用于回顾性分析体重情况和肥胖风险因素。145例患者通过明斯特/海德堡技能量表（FMH）测量生活质量（QoL），77例患者通过儿童生活质量量表（PEDQOL）问卷进行测量。
185例患者中有82例（44%）出现严重肥胖（体重指数[BMI]>3个标准差）。将肥胖患者与79例体重正常（BMI<2个标准差）的患者进行比较。在性别分布、诊断年龄和随访时间方面，肥胖患者与体重正常患者之间未发现差异。然而，发生肥胖的患者在诊断时的BMI标准差分值（SDS）高于未发生肥胖的患者（p<0.0001）。此外，肥胖患者的肿瘤更大（p<0.05），需要分流的脑积水发生率更高（p<0.05），下丘脑受累情况更多（p<0.05）。发生严重肥胖患者的母亲在诊断时BMI更高（p<0.001）。与体重正常患者相比，肥胖患者在诊断时（p<0.05）以及最后一次随访时（p<0.05）的身高标准差分值更高。基于以下风险因素通过逻辑回归计算出颅咽管瘤后严重肥胖的预测模型：患者诊断时BMI>2个标准差（p<0.05；比值比：16.4）、下丘脑受累（p<0.05；比值比：3.4）以及母亲BMI>25kg/m²（p<0.05；比值比：4.6）。术后早期尤其是诊断后的前三年BMI显著增加（p<0.001）。FMH百分位数与BMI SDS呈负相关（斯皮尔曼r：-0.37；p<0.001）。与同年龄组的健康儿童相比，患有颅咽管瘤的儿童在身体能力、认知功能和社会功能方面对其生活质量的评价更为负面（p<0.05）。与非肥胖患者相比，患有颅咽管瘤的严重肥胖患者在除自主性、认知和家庭融合之外的所有领域对其生活质量的估计更低（p<0.05）。
下丘脑肿瘤受累和肥胖的家族倾向是颅咽管瘤患者发生严重肥胖的风险因素。由于诊断后早期即开始体重增加且严重肥胖会导致生活质量下降，对于有风险的患者应考虑早期治疗措施。为了证实我们的结果，启动了关于儿童和青少年颅咽管瘤的前瞻性多中心研究“颅咽管瘤2000”（www.kraniopharyngeom.com）。
