Outcome of Partial Adrenalectomy in MEN2 Syndrome: Personal Experience and Systematic Review of Literature

Author:

Procopio Priscilla Francesca12ORCID,Pennestrì Francesco12ORCID,De Crea Carmela13,Voloudakis Nikolaos12,Bellantone Rocco12,Raffaelli Marco12ORCID

Affiliation:

1. Centro di Ricerca in Chirurgia delle Ghiandole Endocrine e dell’Obesità, Università Cattolica del Sacro Cuore, 00168 Roma, Italy

2. U.O.C. Chirurgia Endocrina e Metabolica, Centro Dipartimentale di Chirurgia Endocrina e dell’Obesità, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy

3. U.O.C. Chirurgia Endocrina, Fatebenefratelli Isola Tiberina-Gemelli Isola, 00186 Roma, Italy

Abstract

Background: Partial adrenalectomy (PA) is an alternative option to total adrenalectomy for the treatment of hereditary pheochromocytoma (PHEO) to preserve cortical function and avoid life-long steroid replacement. The aim of this review is to summarize current evidence in terms of clinical outcome, recurrence, and corticosteroid therapy implementation after PA for MEN2-PHEOs. Material and Methods: From a total of 931 adrenalectomies (1997–2022), 16 of the 194 patients who underwent surgical treatment of PHEO had MEN2 syndrome. There were six patients scheduled for PA. MEDLINE®, EMBASE®, Web of Science, and Cochrane Library were searched for English studies from 1981 to 2022. Results: Among six patients who underwent PA for MEN2-related PHEO in our center, we reported two with bilateral synchronous disease and three with metachronous PHEOs. One recurrence was registered. Less than 20 mg/day Hydrocortison therapy was necessary in 50% of patients after bilateral procedures. Systematic review identified 83 PA for MEN2-PHEO. Bilateral synchronous PHEO, metachronous PHEO and disease recurrence were reported in 42%, 26%, and 4% of patients, respectively. Postoperative steroid implementation was necessary in 65% of patients who underwent bilateral procedures. Conclusions: PA seems to be a safe and valuable option for the treatment of MEN2-related PHEOs, balancing the risk of disease recurrence with the need for corticosteroid therapy.

Publisher

MDPI AG

Subject

Paleontology,Space and Planetary Science,General Biochemistry, Genetics and Molecular Biology,Ecology, Evolution, Behavior and Systematics

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Primary hyperaldosteronism: the choice in favor of conservative surgery;Clinical Medicine (Russian Journal);2023-12-12

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3