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小细胞肺癌伴副肿瘤性库欣综合征的病例系列

Case-series of paraneoplastic Cushing syndrome in small-cell lung cancer.

作者信息

Richa Carine Ghassan, Saad Khadija Jamal, Halabi Georges Habib, Gharios Elie Mekhael, Nasr Fadi Louis, Merheb Marie Tanios

机构信息

Department of Endocrinology, Mount Lebanon Hospital, Beirut, Lebanon.

Lebanese University, Hadath, Lebanon.

出版信息

Endocrinol Diabetes Metab Case Rep. 2018 Mar 8;2018. doi: 10.1530/EDM-18-0004. eCollection 2018.

Abstract

UNLABELLED

The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. The latter is present in two out of three cases and in the remaining one, primary tumor could not be localized, representing a small fraction of patients with paraneoplastic Cushing. Diagnosis is established in the setting of high clinical suspicion by documenting an elevated cortisol level, ACTH and doing dexamethasone suppression test. Treatment options include management of the primary tumor by surgery and chemotherapy and treating Cushing syndrome. Prognosis is poor in SCLC. We concluded that in front of a high clinical suspicion, ectopic Cushing syndrome diagnosis should be considered, and identification of the primary tumor is essential.

LEARNING POINTS

Learning how to suspect ectopic Cushing syndrome and confirm it among all the causes of excess cortisol.Distinguish between occult and severe ectopic Cushing syndrome and etiology.Providing the adequate treatment of the primary tumor as well as for the cortisol excess.Prognosis depends on the differentiation and type of the primary malignancy.

摘要

未标注

本研究的目的是报告三例副肿瘤性或异位库欣综合征病例,这是一种罕见的促肾上腺皮质激素(ACTH)依赖性库欣综合征现象。除了相关文献综述外,还报告了三例病例的临床表现、诊断和治疗情况。结果表明,异位ACTH分泌可与不同类型的肿瘤相关,其中最常见的是支气管类癌肿瘤,其生长缓慢、分化良好,预后良好;以及小细胞肺癌,其分化不良,预后较差。后一种情况在三例病例中有两例出现,另一例中无法定位原发性肿瘤,这代表了副肿瘤性库欣患者中的一小部分。在高度临床怀疑的情况下,通过记录皮质醇水平升高、ACTH水平升高并进行地塞米松抑制试验来确立诊断。治疗选择包括通过手术和化疗治疗原发性肿瘤以及治疗库欣综合征。小细胞肺癌的预后较差。我们得出结论,在高度临床怀疑的情况下,应考虑异位库欣综合征的诊断,并且识别原发性肿瘤至关重要。

学习要点

了解如何在所有皮质醇过多的原因中怀疑并确认异位库欣综合征。区分隐匿性和严重异位库欣综合征及其病因。对原发性肿瘤以及皮质醇过多进行适当治疗。预后取决于原发性恶性肿瘤的分化程度和类型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6942/5843798/99d86eed2ce1/edmcr-2018-180004-g001.jpg

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