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肺类癌瘤所致严重异位促肾上腺皮质激素综合征:一例报告及文献复习

Severe Ectopic Adrenocorticotropic Hormone Syndrome Due to Pulmonary Carcinoid Tumor: A Case Report and Literature Review.

作者信息

Aldrete Karissa, Shahla Leena

机构信息

Department of Endocrinology, Diabetes, & Metabolism, University of Arizona College of Medicine, Phoenix, Arizona.

Division of Endocrinology, Department of Medicine, Duke University, Durham, North Carolina.

出版信息

AACE Clin Case Rep. 2024 Aug 13;10(6):232-235. doi: 10.1016/j.aace.2024.08.002. eCollection 2024 Nov-Dec.

Abstract

BACKGROUND/OBJECTIVE: Pulmonary carcinoid tumors are a rare cause of Cushing's syndrome and usually present with an indolent course. Here, we present a case of rapid onset and severe Cushing's syndrome due to a typical pulmonary carcinoid tumor.

CASE REPORT

A 32-year-old woman developed diabetes, hypertension, and weight gain of 50 pounds over 3 months. Laboratory evaluation was significant for elevated cortisol and adrenocorticotropic hormone levels and levels were nonsuppressible on low and high-dose dexamethasone suppression tests. Chest computed tomography revealed a pulmonary nodule and biopsy showed a typical carcinoid tumor. She was treated with steroidogenesis inhibitors with a plan for surgical excision but developed worsening complications of hypercortisolemia. She eventually underwent cryoablation of the tumor, but unfortunately passed away just 6 months after her initial presentation.

DISCUSSION

Cushing's syndrome in typical pulmonary carcinoid tumors is rarely seen and usually presents with mild hypercortisolism similar to Cushing's disease. Severe hypercortisolemia from typical pulmonary carcinoid tumors can represent a more aggressive pathology or metastatic disease. Severe Cushing's syndrome is associated with significant morbidity and mortality and requires rapid tumor localization as surgical resection can be curative.

CONCLUSION

This case highlights a rare presentation of severe Cushing's syndrome due to a typical pulmonary carcinoid.

摘要

背景/目的:肺类癌瘤是库欣综合征的罕见病因,通常病程进展缓慢。在此,我们报告一例因典型肺类癌瘤导致的快速起病且严重的库欣综合征病例。

病例报告

一名32岁女性在3个月内出现糖尿病、高血压,体重增加50磅。实验室检查显示皮质醇和促肾上腺皮质激素水平升高,且在低剂量和高剂量地塞米松抑制试验中水平均不可被抑制。胸部计算机断层扫描显示一个肺结节,活检显示为典型类癌瘤。她接受了类固醇生成抑制剂治疗,并计划进行手术切除,但出现了高皮质醇血症的恶化并发症。她最终接受了肿瘤冷冻消融治疗,但不幸的是,在初次就诊仅6个月后就去世了。

讨论

典型肺类癌瘤导致的库欣综合征很少见,通常表现为与库欣病相似的轻度皮质醇增多症。典型肺类癌瘤引起的严重高皮质醇血症可能代表更具侵袭性的病理改变或转移性疾病。严重的库欣综合征与显著的发病率和死亡率相关,由于手术切除可能治愈,因此需要快速定位肿瘤。

结论

本病例突出了典型肺类癌导致严重库欣综合征的罕见表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ed8/11680749/11ddd606445a/gr1.jpg

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