Context: Quality of survival (QoS) is frequently impaired in childhood-onset craniopharyngioma (CP) patients due to sequelae caused by the hypothalamic syndrome. The debate, whether primary hypothalamic involvement (HI) has apriori prognostic impact or surgical hypothalamic lesions (HL) determine outcome, is controversial. Accordingly, we analyzed, whether CP patients at high risk for hypothalamic obesity due to primary HI of anterior and posterior hypothalamic structures benefit from hypothalamus-sparing surgical strategy.
Patients and methods: We included 109 CP patients with reference-confirmed initial anterior and posterior HI recruited between 2007 and 2014 in KRANIOPHARYNGEOM 2007. Progression-free survival (PFS), body mass index (BMI), QoS as assessed by Pediatric Quality of Life (PEDQOL) questionnaire, and functional capacity (FMH ability scale) were analyzed one and 3 years after CP diagnosis and at last follow-up visit with regard to the degree of reference-confirmed surgical HL.
Results: Surgical HL were reference-confirmed in 86 of 109 (79%) CP patients included in our study (23 no HL, 29 anterior HL, 57 anterior plus posterior HL). PFS and BMI at diagnosis were similar in our CP subgroups with different degree of HL. Significant increases in BMI occurred in all HL subgroups during follow-up (median follow-up interval at last visit: 6.1 yrs, range: 3.010.3 yrs). However, CP with anterior plus posterior HL presented with higher BMI at the time points 1 yr after diagnosis (median BMI: +5.21 SD, range: −0.32 to 13.09 S.D.) and at last visit (median BMI: +5.74 S.D., range: −0.82 to 14.65 S.D.), when compared to patients without HL (median BMI at 1 yr follow-up: +1.72 S.D., range: −0.07 to 10.37 S.D., P=0.001; at last visit: +2.27 S.D., range: −1.77 6.99 S.D., P<0.001) and compared to patients with anterior HL (median BMI at 1 yr follow-up: +2.46 S.D., range: −1.86 to 10.37 S.D., P=0.002; at last visit: +2.87 SD, range: −0.81 to 11.11 S.D., P=0.001). QoS was better during follow-up in CP without HL for the PEDQOL domains physical function (P=0.047), emotional stability (P=0.040), and social functionality family (P=0.002) when compared to CP patients with anterior plus posterior HL. Differences in terms of functional capacity did not reach statistical significance with regard to HL.
Conclusions: Hypothalamus-sparing surgical strategy does not result in increased relapse and progression rates, improves QoS and ameliorates the development of severe obesity also in CP patients at high apriori risk for hypothalamic obesity due to primary presurgical HI.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology