Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma.

We analyzed whether childhood craniopharyngioma predisposes to obesity and growth impairment. Height/length, body mass index (BMI), and hypothalamic involvement were evaluated in 90 patients at standardized ages and time points before, after, and at the time of diagnosis. Relevant decreases in height sd score (SDS) started at 10-12 months of age and persisted until diagnosis of childhood craniopharyngioma. Relevant increases in BMI SDS were detectable between 4 and 5 yr of age. Postoperative BMI SDS (yr 1-6) had a weak positive correlation with BMI SDS at the time of diagnosis. In linear regression analysis, hypothalamic tumor involvement (P < 0.001), ponderal index at birth (P = 0.014), and BMI SDS at age 6-7 months (P = 0.029) and at age 5 yr (P < 0.001) had relevant and independent impacts on the development of obesity. Patients with hypothalamic involvement (n = 48) presented lower ponderal index and BMI SDS at birth and higher BMI SDS at the time of diagnosis (P < 0.001) as well as during annual follow-up (P < 0.001) compared with patients without hypothalamic involvement (n = 42). From childhood (3.5-4 yr) to the time of diagnosis, growth rates were reduced for patients with hypothalamic tumor involvement. Patients without hypothalamic involvement presented reduced growth rates in early infancy (age 10-12 months) that persisted until diagnosis. We conclude that reduced growth rates occur quite early in history; BMI SDS increases occur later and are predictive of obesity. Hypothalamic involvement is the major risk factor for obesity in patients with childhood craniopharyngioma.

Current strategies in diagnostics and endocrine treatment of patients with childhood craniopharyngioma during follow-up--recommendations in KRANIOPHARYNGEOM 2000.

Craniopharyngiomas are rare dysontogenetic malformations. As the survival rate after craniopharyngioma diagnosed during childhood and adolescence is high, prognosis and quality of life (QoL) in survivors mainly depend on adverse late effects such as hypopituitarism and obesity. Appropriate laboratory diagnostics of endocrine deficiencies and sufficient hormonal substitution have significant impact on prognosis and QoL. In order to evaluate and standardize diagnostic and therapeutical strategies in childhood craniopharyngioma the prospective multicenter surveillance study KRANIOPHARYNGEOM 2000 was initiated for patients diagnosed with craniopharyngioma during childhood and adolescence. We are reporting on current strategies for laboratory diagnostics and endocrine substitution in patients with childhood craniopharyngioma recruited in KRANIOPHARYNGEOM 2000.

Functional capacity and body mass index in patients with sellar masses--cross-sectional study on 403 patients diagnosed during childhood and adolescence.

RATIONALE: We analyzed the impact of tumour localization and histology on functional capacity (FC) and body mass index (BMI) in children with sellar masses. METHODS: FC was evaluated using the ability scale Fertigkeitenskala Münster-Heidelberg in 403 children and adolescents with sellar masses (276 craniopharyngioma, 14 germinoma, 21 optic/chiasmatic glioma, 40 hypothalamic glioma, 13 cysts of Rathke's cleft and 39 other sellar masses). Besides tumour localization, the influence of gender, irradiation and age at diagnosis and at evaluation on FC and BMI was analyzed. General linear models with explanatory influential variables were built. RESULTS: In multivariate analysis, only age at diagnosis (p<0.001) and hypothalamic involvement (p=0.005) had relevant impact on FC. The second model showed BMI at diagnosis (p<0,001), hypothalamic involvement (p<0.001) and craniopharyngioma (p=0,004) to influence BMI at the latest evaluation. CONCLUSION: We conclude that hypothalamic involvement and young age at diagnosis had major impact on FC and BMI and should be considered as risk factors for impaired rehabilitation.

Longitudinal study on quality of life in 102 survivors of childhood craniopharyngioma.

OBJECTS: We longitudinally analysed functional capacity and quality of life (QoL) in 102 patients with a childhood craniopharyngioma during follow-up. METHODS: The Fertigkeitenskala Münster-Heidelberg (FMH) ability scale was used for QoL assessment. Multivariate analysis evaluated FMH scores at various time points, examining hypothalamus involvement (HI), body mass index (BMI), degree of resection, tumour progression, relapse and irradiation. RESULTS AND CONCLUSION: Patients without HI (n=60) self-assessed higher QoL at baseline (p=0.001) and follow-up (p<0.001) than patients with HI (n=42). Only patients without HI evaluated at baseline >12 months after diagnosis showed longitudinal improvement in FMH scores (p=0.040). Rates of incomplete resection and irradiation were higher (p=0.070 and p=0.002 respectively) in patients with HI. In multivariate analysis, only HI, tumour relapse, progression, baseline FMH score, and time between diagnosis and baseline evaluation had independent impact on QoL. HI, tumour progression, and relapse had long-term QoL affects-most notably, severe obesity.