Background: Paediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathkes pouch (CRP) frequently suffer disease- and treatment-related sequelae.
Objective and hypotheses: We analysed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP.
Method: Using retrospective analysis of patient records, presence of initial HY or HI was assessed in 177 pediatric patients (163 CP, 14 CRP). 20-years overall survival (OS) and progression-free survival (PFS), FC, and BMI were analysed with regard to initial HY, degree of resection, or HI.
Results: 105 patients (103/163 CP, 2/14 CRP) presented with initial HY and 96 presented with HI. HY at diagnosis was associated (P=0.000) with papilledema, neurological deficits, and higher BMI at diagnosis and during follow-up. OS, PFS, and FC were not affected by HY at initial diagnosis. HI at diagnosis (96/177) had major negative impact on long-term prognosis. Sellar masses with HI were associated with lower OS (0.84±0.04; P=0.021), lower FC (P=0.003), and higher BMI at diagnosis and last follow-up (P=0.000) when compared with sellar masses without HI (OS: 0.94±0.05). PFS was not affected by HI or degree of resection.
Conclusion: Initial HY has no impact on outcome in patients with sellar masses. OS and FC are impaired in survivors presenting with initial HI. PFS is not affected by HY, HI, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial HI to prevent further hypothalamic damage.
Funding: German Childhood Cancer Foundation, Bonn, Germany.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology