Survival probabilities in patients with ectopic Cushing's syndrome - a systematic review and a single-arm meta-analysis.

IF 5.3 1区医学 Q1 ENDOCRINOLOGY & METABOLISM

European Journal of Endocrinology Pub Date : 2025-06-02 DOI:10.1093/ejendo/lvaf114

Marta Piasecka, Eleni Papakokkinou, Adam Piasecki, Henrik Falhammar, Oskar Ragnarsson

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引用次数: 0

Abstract

Objective: We aimed to estimate 1- and 5-year survival probabilities in patients with different forms of ectopic Cushing's syndrome (ECS) and identify factors influencing survival.

Methods: In this systematic review and meta-analysis, we searched the online databases PubMed, Scopus and Web of Science up to October 18th, 2023, for studies reporting survival in patients with ECS. Data extraction and risk of bias assessment were performed by three independent investigators. Primary outcome was survival in patients with ECS and secondary outcome was factors influencing survival.

Results: We included 40 studies with a total of 1148 patients. The pooled mean 1-year survival probability for ECS of mixed etiologies was 78% while the mean pooled 5-year survival probability was 47%. The 5-year survival probabilities for patients with pulmonary neuroendocrine neoplasm (NEN) was 81%, occult ECS 66%, thymic NEN 50% and pancreatic NEN 40%. Only eight studies reported factors influencing survival, where total resection of the primary tumor was associated with better overall survival, and unresectable tumors, metastatic disease at diagnosis, severe hypercortisolism, hypokalemia, and new onset diabetes mellitus were associated with worse prognosis.

Conclusion: Survival in ECS varies considerably, mainly due to the underlying origin of the tumor, tumor stage and severity of the hypercortisolism. Further studies analyzing the importance of factors affecting survival are needed.

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异位库欣综合征患者的生存概率——一项系统综述和单臂荟萃分析。

目的：我们旨在评估不同形式异位库欣综合征（ECS）患者的1年和5年生存率，并确定影响生存率的因素。方法：在本系统综述和荟萃分析中，我们检索了截至2023年10月18日的在线数据库PubMed、Scopus和Web of Science，以获取报告ECS患者生存的研究。数据提取和偏倚风险评估由三名独立调查人员进行。ECS患者的主要结局是生存，次要结局是影响生存的因素。结果：我们纳入了40项研究，共1148例患者。混合病因的ECS 1年平均生存率为78%，5年平均生存率为47%。肺神经内分泌肿瘤（NEN）患者的5年生存率为81%，隐匿性ECS为66%，胸腺NEN为50%，胰腺NEN为40%。只有8项研究报告了影响生存的因素，其中原发肿瘤的完全切除与更好的总生存相关，而不可切除的肿瘤、诊断时的转移性疾病、严重的高皮质醇血症、低钾血症和新发糖尿病与更差的预后相关。结论：ECS患者的生存率差异很大，主要与肿瘤的潜在起源、肿瘤分期和高皮质醇血症的严重程度有关。需要进一步研究分析影响生存因素的重要性。

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

European Journal of Endocrinology 医学-内分泌学与代谢

CiteScore

9.80

自引率

3.40%

发文量

354

审稿时长

1 months

期刊介绍： European Journal of Endocrinology is the official journal of the European Society of Endocrinology. Its predecessor journal is Acta Endocrinologica. The journal publishes high-quality original clinical and translational research papers and reviews in paediatric and adult endocrinology, as well as clinical practice guidelines, position statements and debates. Case reports will only be considered if they represent exceptional insights or advances in clinical endocrinology. Topics covered include, but are not limited to, Adrenal and Steroid, Bone and Mineral Metabolism, Hormones and Cancer, Pituitary and Hypothalamus, Thyroid and Reproduction. In the field of Diabetes, Obesity and Metabolism we welcome manuscripts addressing endocrine mechanisms of disease and its complications, management of obesity/diabetes in the context of other endocrine conditions, or aspects of complex disease management. Reports may encompass natural history studies, mechanistic studies, or clinical trials. Equal consideration is given to all manuscripts in English from any country.