Predictors of Mortality in Patients With Multiple Endocrine Neoplasia Type 1

IF 2.4 3区医学 Q2 ENDOCRINOLOGY & METABOLISM

Clinical Endocrinology Pub Date : 2025-05-02 DOI:10.1111/cen.15257

Jasmine J. Zhu, John R Burgess

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引用次数: 0

Abstract

Objective

Multiple Endocrine Neoplasia Type 1 (MEN 1) is an autosomal dominant disease predisposing to hyperplasia and neoplasia in diverse endocrine tissues. Patients typically present with endocrine abnormalities before the age of 30 years and have reduced life expectancy. Our objective was to determine predictors of premature mortality in MEN 1.

Design

Tertiary hospital based retrospective cohort study.

Patients

One hundred and thirty patients with a common MEN1 genotype.

Measurements

Kaplan-Meier survival analysis of median life expectancy (MLE).

Results

The overall cohort MLE was 70.8 years. Sex and year of birth were not predictive of survival. A diagnosis before age 45 years of adrenal nodularity (MLE 51.8 years), hypergastrinaemia (MLE 66.2 years), or liver lesions (MLE 38.6) were associated with a significant reduction in survival (26.2 years, p < 0.01, 6.4 years, p = 0.03, and 30.3 years, p < 0.01 respectively) compared to being diagnosed with these conditions later in life. In contrast, diagnosis before age 45 years of pancreatic nodularity (MLE 68.9 years) and primary hyperparathyroidism (MLE 68.9 years) were not predictive of survival.

Conclusion

Patients with MEN 1 diagnosed before age 45 with adrenal nodularity, hypergastrinaemia or liver lesions had significantly reduced survival. The explanation for non-secretory and benign adrenal macronodular hyperplasia being associated with diminished life expectancy is unclear.

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1型多发性内分泌肿瘤患者死亡率的预测因素。

目的：多发性内分泌肿瘤1型（MEN 1）是一种常染色体显性遗传病，易发生于多种内分泌组织的增生和肿瘤。患者通常在30岁前出现内分泌异常，预期寿命缩短。我们的目的是确定男性1期过早死亡的预测因素。设计：基于三级医院的回顾性队列研究。患者：130例常见MEN1基因型患者。测量方法：Kaplan-Meier生存分析中位预期寿命（MLE）。结果：总体队列MLE为70.8岁。性别和出生年份不能预测生存率。45岁前诊断为肾上腺结节（MLE为51.8年）、高胃泌素血症（MLE为66.2年）或肝脏病变（MLE为38.6年）与生存率显著降低（26.2年，p）相关。结论：45岁前诊断为男性1型患者的肾上腺结节、高胃泌素血症或肝脏病变显著降低生存率。非分泌性和良性肾上腺大结节增生与预期寿命缩短有关的原因尚不清楚。

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

Clinical Endocrinology 医学-内分泌学与代谢

CiteScore

6.40

自引率

3.10%

发文量

192

审稿时长

1 months

期刊介绍： Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.