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First page of “Craniopharyngioma (CP), Rathke cleft cyst (RCC) and empty sella (ES) natural history and endocrine outcome in pediatric age: a single tertiary centre experience over the 2000-2024 period”

Introduction: Craniopharyngioma (CP), Rathke Cleft Cyst (RCC) and Empty Sella (ES) are clinical conditions that may lead to endocrine disorders. Methods: Clinical data of all pediatric patients aged 0-18 years with diagnosis of CP, RCC or ES, referred to our department over the period 2000-2024 were analysed. Results: Data of 13 subjects with CP, 35 subjects with RCC and 32 patients with ES were analysed. Mean age at diagnosis was 6.6 ± 1.2 years in the CP group, 8.45 ± 3.2 years in the RCC group, and 10.55 ± 2.1 years in the ES group (p = 0.03). Prior endocrine disorder was the reason for requesting MRI in 27/80 patients: 1/13 of patients with CP, 16/35 of those with RCC, and 10/32 of patients with ES (p = 0.04). Among RCC patients, higher age and RCC size were observed in subjects with endocrine disorders (p = 0.04). Similar trend was observed in patients with ES, with significantly higher age in those with endocrine disorders compared to those without (p = 0.04). During follow-up, endocrine disorders were diagnosed in all patients with CP (13/13; 100%), in 5 out of 20 patients with RCC (25%), and in none of the patients with ES. Conclusion: CP, RCC and ES need baseline and over a prolonged period of time endocrinological and neuroradiological follow-up, in order to detect promptly endocrine defects and sellar/suprasellar region alterations and multidisciplinary follow-up in tertiary centres is mandatory.

S. Karger AG, Basel
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2025
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