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1型多发性内分泌腺瘤病综合征中功能性胸腺神经内分泌肿瘤所致库欣综合征

Cushing's Syndrome Due to a Functional Thymic Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Syndrome.

作者信息

Asemota Iriagbonse R, Ajiboye Oyintayo, Nwaichi Chineme, Mbachi Chimezie, Mba Benjamin

机构信息

Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA.

出版信息

Cureus. 2021 Oct 7;13(10):e18590. doi: 10.7759/cureus.18590. eCollection 2021 Oct.

Abstract

Multiple endocrine neoplasia type 1 (MEN 1) syndrome is characterized by endocrinopathies and could be associated with thymic neuroendocrine tumors (NET). On rare occasions, they can be functional adrenocorticotropic hormone-secreting thymic carcinoid leading to Cushing's syndrome. In this report, we describe a case of adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome due to a thymic NET associated with MEN type 1 syndrome. We highlight its aggressive clinical course, the premise for a high index of suspicion for an ectopic ACTH secretion, and the need for early surgical resection combined with medical therapy and alternative treatments.

摘要

1型多发性内分泌腺瘤病(MEN 1)综合征的特征为内分泌病变,可能与胸腺神经内分泌肿瘤(NET)有关。在极少数情况下,它们可能是分泌促肾上腺皮质激素的功能性胸腺类癌,导致库欣综合征。在本报告中,我们描述了一例因与MEN 1综合征相关的胸腺NET引起的促肾上腺皮质激素(ACTH)依赖性库欣综合征病例。我们强调了其侵袭性的临床病程、对异位ACTH分泌高度怀疑的依据,以及早期手术切除联合药物治疗和替代治疗的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec58/8572323/cf24562224be/cureus-0013-00000018590-i01.jpg

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