Ectopic adrenocorticotropic hormone syndrome due to olfactory neuroblastoma: A case report and literature review

IF 1.8 4区医学 Q4 ENDOCRINOLOGY & METABOLISM

Endocrinologia Diabetes Y Nutricion Pub Date : 2025-06-01 DOI:10.1016/j.endien.2025.501576

Mónica Baptista Lopes , Ricardo Fonseca , João Fernandes Serôdio , Ricardo Paquete Oliveira , José Delgado Alves

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引用次数: 0

Abstract

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a cause of Cushing's syndrome usually associated with neuroendocrine tumors. Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of the olfactory epithelium. This is the case of a 56-year-old woman with an ONB presenting with EAS. After initiating metyrapone, she developed a Pneumocystis jirovecii pneumonia. Following successful treatment of the infection, she underwent surgical tumor excision and radiotherapy, which has been in remission for the past 3 years. The authors provide a literature review of the 30 previously published cases of ONB presenting with EAS. Most were reported in middle-aged men, with a recurrence rate of 15.6% (3 patients eventually died). A total of 9.5% of all reported had an infection after starting corticosteroid-blocking therapy. ONB is a very rare cause of EAS with poor prognosis and a relapsing course. In the presence of severe hypercortisolism, chemoprophylaxis for common opportunistic agents must be considered.

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嗅觉神经母细胞瘤所致异位促肾上腺皮质激素综合征1例报告并文献复习

异位促肾上腺皮质激素（ACTH）综合征（EAS）是库欣综合征的病因之一，通常与神经内分泌肿瘤相关。嗅觉神经母细胞瘤是一种罕见的嗅觉上皮恶性肿瘤。这是一名56岁的女性ONB，表现为EAS。在使用美替拉酮后，她出现了乙氏肺囊虫肺炎。在成功治疗感染后，她接受了手术肿瘤切除和放疗，在过去的3年里病情一直缓解。作者提供了一个文献综述的30例先前发表的ONB表现为EAS。多数为中年男性，复发率为15.6%（最终死亡3例）。在所有报告的患者中，有9.5%的人在开始皮质类固醇阻断治疗后发生了感染。ONB是一种非常罕见的病因，预后差，易复发。在存在严重的高皮质醇血症时，必须考虑对常见的机会性药物进行化学预防。

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

Endocrinologia Diabetes Y Nutricion Multiple-

CiteScore

2.10

自引率

10.50%

发文量

期刊介绍： Endocrinología, Diabetes y Nutrición is the official journal of the Spanish Society of Endocrinology and Nutrition (Sociedad Española de Endocrinología y Nutrición, SEEN) and the Spanish Society of Diabetes (Sociedad Española de Diabetes, SED), and was founded in 1954. The aim of the journal is to improve knowledge and be a useful tool in practice for clinical and laboratory specialists, trainee physicians, researchers, and nurses interested in endocrinology, diabetes, nutrition and related disciplines. It is an international journal published in Spanish (print and online) and English (online), covering different fields of endocrinology and metabolism, including diabetes, obesity, and nutrition disorders, as well as the most relevant research produced mainly in Spanish language territories. The quality of the contents is ensured by a prestigious national and international board, and by a selected panel of specialists involved in a rigorous peer review. The result is that only manuscripts containing high quality research and with utmost interest for clinicians and professionals related in the field are published. The Journal publishes Original clinical and research articles, Reviews, Special articles, Clinical Guidelines, Position Statements from both societies and Letters to the editor. Endocrinología, Diabetes y Nutrición can be found at Science Citation Index Expanded, Medline/PubMed and SCOPUS.