Fulminant ectopic Cushing's syndrome caused by metastatic small intestine neuroendocrine tumour - a case report and review of the literature.

IF 1.3 4区医学 Q4 GASTROENTEROLOGY & HEPATOLOGY

Acta gastro-enterologica Belgica Pub Date : 2024-01-01 DOI:10.51821/87.1.11872

B Alliet, C Severi, T Veekmans, J Cuypers, H Topal, C M Deroose, T Roskams, M Bex, J Dekervel

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引用次数: 0

Abstract

Cushing's syndrome (CS) secondary to adrenocorticotropic hormone (ACTH) producing tumours is a severe condition with a challenging diagnosis. Ectopic ACTH-secretion often involves neuroendocrine tumours (NET) in the respiratory tract. ACTH-secreting small intestine neuro-endocrine tumours (siNET) are extremely rare entities barely reported in literature. This review is illustrated by the case of a 75-year old woman with fulminant ectopic CS caused by a ACTH-secreting metastatic siNET. Severe hypokalemia, fluid retention and refractory hypertension were the presenting symptoms. Basal and dynamic laboratory studies were diagnostic for ACTH-dependent CS. Extensive imaging studies of the pituitary and thorax-abdomen areas were normal, while [68Ga]Ga-DOTATATE PET-CT revealed increased small intestine uptake in the left iliac fossa. The hypercortisolism was well controlled with somatostatin analogues, after which a debulking resection of the tumour was performed. Pathological investigation confirmed a well-differentiated NET with sporadic ACTH immunostaining and post-operative treatment with somatostatin analogues was continued with favourable disease control.

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转移性小肠神经内分泌肿瘤引起的充血性异位库欣综合征--病例报告和文献综述。

继发于促肾上腺皮质激素（ACTH）分泌肿瘤的库欣综合征（CS）是一种严重的疾病，其诊断极具挑战性。异位 ACTH 分泌通常涉及呼吸道的神经内分泌肿瘤（NET）。分泌促肾上腺皮质激素的小肠神经内分泌肿瘤（siNET）极为罕见，文献中鲜有报道。本综述以一名 75 岁女性的病例为例作了说明，她患有由分泌促肾上腺皮质激素的转移性 siNET 引起的暴发性异位 CS。严重的低钾血症、液体潴留和难治性高血压是其主要症状。基础和动态实验室检查诊断为促肾上腺皮质激素依赖性 CS。垂体和胸腹部的广泛影像学检查正常，而[68Ga]Ga-DOTATATE PET-CT显示左侧髂窝的小肠摄取增加。使用体生长激素类似物后，高皮质醇血症得到了很好的控制，随后进行了肿瘤切除术。病理检查证实这是一种分化良好的母细胞瘤，伴有散发性促肾上腺皮质激素免疫染色，术后继续使用体生长激素类似物治疗，病情得到了良好控制。

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来源期刊

Acta gastro-enterologica Belgica Medicine-Gastroenterology

CiteScore

2.30

自引率

20.00%

发文量

期刊介绍： The Journal Acta Gastro-Enterologica Belgica principally publishes peer-reviewed original manuscripts, reviews, letters to editors, book reviews and guidelines in the field of clinical Gastroenterology and Hepatology, including digestive oncology, digestive pathology, as well as nutrition. Pure animal or in vitro work will not be considered for publication in the Journal. Translational research papers (including sections of animal or in vitro work) are considered by the Journal if they have a clear relationship to or relevance for clinical hepato-gastroenterology (screening, disease mechanisms and/or new therapies). Case reports and clinical images will be accepted if they represent an important contribution to the description, the pathogenesis or the treatment of a specific gastroenterology or liver problem. The language of the Journal is English. Papers from any country will be considered for publication. Manuscripts submitted to the Journal should not have been published previously (in English or any other language), nor should they be under consideration for publication elsewhere. Unsolicited papers are peer-reviewed before it is decided whether they should be accepted, rejected, or returned for revision. Manuscripts that do not meet the presentation criteria (as indicated below) will be returned to the authors. Papers that go too far beyond the scope of the journal will be also returned to the authors by the editorial board generally within 2 weeks. The Journal reserves the right to edit the language of papers accepted for publication for clarity and correctness, and to make formal changes to ensure compliance with AGEB’s style. Authors have the opportunity to review such changes in the proofs.