"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".

in Neuroendocrinology by Gerdi Tuli, Jessica Munarin

TLDR

  • The study analyzed pediatric patients with CP, RCC, or ES and found that prior endocrine disorder was a reason for requesting MRI in 27/80 patients, with a higher incidence in RCC and ES compared to CP. During follow-up, endocrine disorders were diagnosed in all CP patients, 25% of RCC patients, and none of the ES patients.

Abstract

Craniopharyngioma (CP), Rathke Cleft Cyst (RCC) and Empty Sella (ES) are clinical conditions that may lead to endocrine disorders. 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. 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. 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.

Overview

  • The study analyzed clinical data of pediatric patients (0-18 years) with Craniopharyngioma (CP), Rathke Cleft Cyst (RCC), or Empty Sella (ES) referred to a department from 2000-2024.
  • The data included 13 CP, 35 RCC, and 32 ES patients, with mean ages at diagnosis ranging from 6.6 to 10.55 years (p = 0.03).
  • The study aimed to investigate the prevalence and characteristics of endocrine disorders in these patients and the need for baseline and longitudinal endocrinological and neuroradiological follow-up.

Comparative Analysis & Findings

  • Prior endocrine disorder was the reason for requesting MRI in 27/80 patients, with a higher incidence in RCC (16/35) and ES (10/32) compared to CP (1/13) patients (p = 0.04).
  • Among RCC patients, higher age and RCC size were observed in subjects with endocrine disorders (p = 0.04).
  • During follow-up, endocrine disorders were diagnosed in all CP patients (100%), 25% of RCC patients, and none of the ES patients.

Implications and Future Directions

  • The study highlights the importance of multidisciplinary follow-up at tertiary centers to detect endocrine defects and sellar/suprasellar region alterations in patients with CP, RCC, or ES.
  • Further research is needed to investigate the relationship between the size and age of RCCs and ES, as well as the potential impact of endocrine disorders on patient outcomes.
  • Longitudinal studies are recommended to evaluate the predictors and long-term consequences of endocrine disorders in these patient populations.
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