Asemota Iriagbonse R, Ajiboye Oyintayo, Nwaichi Chineme, Mbachi Chimezie, Mba Benjamin
Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA.
Cureus. 2021 Oct 7;13(10):e18590. doi: 10.7759/cureus.18590. eCollection 2021 Oct.
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.
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。