Neuroendocrinology · 2025
Craniopharyngioma, Rathke Cleft Cyst, and Empty Sella Natural History and Endocrine Outcome in Pediatric Age: A Single Tertiary Center Experience over the 2000-2024 Period.
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Tuli Gerdi, Munarin Jessica
Аннотация
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 analyzed.
RESULTS: Data of 13 subjects with CP, 35 subjects with RCC, and 32 patients with ES were analyzed. 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 centers is mandatory.
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 analyzed.
RESULTS: Data of 13 subjects with CP, 35 subjects with RCC, and 32 patients with ES were analyzed. 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 centers is mandatory.