直肠大细胞神经内分泌癌引起的异位促肾上腺皮质激素释放激素综合征:罕见病例报告。

IF 3.9 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM
Therapeutic Advances in Endocrinology and Metabolism Pub Date : 2024-12-11 eCollection Date: 2024-01-01 DOI:10.1177/20420188241305026
Yuan Lou, Huan Chen, Si-Jia Fei, Qing-Hua He, Qi Pan
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引用次数: 0

摘要

异位促肾上腺皮质激素释放激素(CRH)综合征是促肾上腺皮质激素依赖性库欣综合征的一种罕见亚型,与多种来源的肿瘤有关。在此,我们报告了一位37岁的女性,被诊断为继发于直肠大细胞神经内分泌癌的异位CRH综合征,这是迄今为止CRH分泌肿瘤中前所未有的部位。患者表现出库欣综合征的典型特征,并有高皮质醇血症和昼夜皮质醇分泌紊乱的实验室证据支持。影像学检查排除垂体腺瘤,而结肠镜检查发现直肠恶性肿瘤。免疫组化染色证实异位CRH综合征的存在。尽管及时开始化疗,但患者的病情迅速恶化,突出了与异位CRH综合征相关的直肠大细胞神经内分泌癌的侵袭性和惨淡预后。该病例强调了早期识别和综合管理对优化患者预后的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Ectopic corticotropin-releasing hormone syndrome caused by rectal large cell neuroendocrine carcinoma: a rare case report.

Ectopic corticotropin-releasing hormone (CRH) syndrome, a rare subtype of adrenocorticotropic hormone-dependent Cushing syndrome, is associated with tumors of diverse origins. Here, we present a case of a 37-year-old female diagnosed with ectopic CRH syndrome secondary to rectal large cell neuroendocrine carcinoma, a hitherto unprecedented site for CRH-secreting tumors. The patient presented with classical features of Cushing syndrome, supported by laboratory evidence of hypercortisolemia and disrupted diurnal cortisol secretion. Imaging studies ruled out a pituitary adenoma, whereas colonoscopy identified a rectal malignancy. Immunohistochemical staining confirmed the presence of ectopic CRH syndrome. Despite prompt chemotherapy initiation, the patient's condition rapidly deteriorated, highlighting the aggressive nature and dismal prognosis associated with rectal large cell neuroendocrine carcinoma linked to ectopic CRH syndrome. This case underscores the importance of early recognition and comprehensive management to optimize patient outcomes.

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来源期刊
Therapeutic Advances in Endocrinology and Metabolism
Therapeutic Advances in Endocrinology and Metabolism Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
7.70
自引率
2.60%
发文量
42
审稿时长
8 weeks
期刊介绍: Therapeutic Advances in Endocrinology and Metabolism delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of endocrinology and metabolism.
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