ECE2016 Eposter Presentations Neuroendocrinology (43 abstracts)
History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis
Anika Hoffmann 1 , Svenja Boekhoff 1 , Ursel Gebhardt 1 , Anthe S Sterkenburg 1, , Anna M Daubenbüchel 1, , Maria Eveslage 3 & Hermann L Müller 1
1Dep. of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany; 2UMCG Groningen, Groningen, The Netherlands; 3Institute of Biostatistics and Clinical Research, University of Münster, Münster, Germany.
Objective: Childhood craniopharyngiomas (CP) are often diagnosed after long duration of history (DOH). Tumor size, hypothalamic involvement (HI), and obesity are associated with reduced overall survival (OS) and functional capacity (FC). The effect of DOH and specific symptoms in history on presentation at initial diagnosis and long-term prognosis are unknown.
Design: Retrospective analysis of patients’ records and prospective longitudinal follow-up.
Methods: Histories of 411 CP patients recruited in HIT Endo, KRANIOPHARYNGEOM 2000 were retrospectively evaluated for DOH, symptoms and characteristics. The effect of specific manifestations and DOH on clinical presentation and tumor characteristics at time of initial CP diagnosis and long-term outcome were analyzed. Main outcome measures were 10-yr OS and progression-free survival (PFS), FC, and body mass index (BMI) during longitudinal follow-up.
Results: Median DOH was 6 mo (range: 0.1–108 mo) and correlated with age at diagnosis. Tumor size, HI, degree of resection, and BMI at diagnosis were not related to DOH. In multivariate analysis adjusted for age at diagnosis, only hydrocephalus was found to have a relevant influence on DOH. Visual and neurological deficits were associated with larger initial tumor size and impaired 10-yr OS. Weight gain and growth failure were observed with longest DOH. PFS and FC were not related to any specific symptom. Endocrine deficits at diagnosis were associated with long DOH.
Conclusions: CP is frequently diagnosed after long DOH, especially in older children. However, DOH was not associated with tumor size, HI, survival or FC. Visual and neurological deficits necessitate rapid diagnostic work-up.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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