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.

IF 3.2 2区医学 Q2 ENDOCRINOLOGY & METABOLISM

Neuroendocrinology Pub Date : 2025-05-19 DOI:10.1159/000546493

Gerdi Tuli, Jessica Munarin

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引用次数: 0

Abstract

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.

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颅咽管瘤（CP）， Rathke裂囊（RCC）和空鞍（ES）的自然史和儿科年龄的内分泌结果：2000-2024年期间的单一三级中心经验。

颅咽管瘤（CP）， Rathke Cleft囊肿（RCC）和空蝶鞍（ES）是可能导致内分泌紊乱的临床病症。方法：分析我院2000-2024年收治的0 ~ 18岁CP、RCC、ES患儿的临床资料。结果：分析了13例CP患者、35例RCC患者和32例ES患者的资料。CP组的平均诊断年龄为6.6±1.2岁，RCC组为8.45±3.2岁，ES组为10.55±2.1岁（p = 0.03）。在27/80的患者中，既往内分泌失调是要求MRI的原因：1/13的CP患者，16/35的RCC患者，10/32的ES患者（p = 0.04）。在RCC患者中，内分泌紊乱患者的年龄和RCC大小均较高（p = 0.04）。在ES患者中也观察到类似的趋势，有内分泌紊乱的患者年龄明显高于无内分泌紊乱的患者（p = 0.04）。随访期间，所有CP患者均被诊断为内分泌紊乱(13/13；100%)， 20例RCC患者中有5例（25%），ES患者中无一例。结论：CP、RCC和ES需要基线和长时间的内分泌和神经影像学随访，以便及时发现内分泌缺陷和鞍上区改变，三级中心的多学科随访是必要的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuroendocrinology 医学-内分泌学与代谢

CiteScore

8.30

自引率

2.40%

发文量

审稿时长

6-12 weeks

期刊介绍： ''Neuroendocrinology'' publishes papers reporting original research in basic and clinical neuroendocrinology. The journal explores the complex interactions between neuronal networks and endocrine glands (in some instances also immunecells) in both central and peripheral nervous systems. Original contributions cover all aspects of the field, from molecular and cellular neuroendocrinology, physiology, pharmacology, and the neuroanatomy of neuroendocrine systems to neuroendocrine correlates of behaviour, clinical neuroendocrinology and neuroendocrine cancers. Readers also benefit from reviews by noted experts, which highlight especially active areas of current research, and special focus editions of topical interest.