异位肢端肥大症的临床和生化谱系

IF 6.1 1区 医学 Q1 ENDOCRINOLOGY & METABOLISM
Patricia Fainstein-Day (Associate Physician, Professor and Investigator) , Tamara Estefanía Ullmann (Associate Physician) , Mercedes Corina Liliana Dalurzo (Associate Physician) , Gustavo Emilio Sevlever (Director, Teaching, Investigator) , David Eduardo Smith (Staff Surgeon)
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引用次数: 0

摘要

异位肢端肥大症是一种罕见的疾病,由垂体外中枢或外周神经内分泌肿瘤(NET)引起,可分泌过多 GH 或更常见的 GHRH。它影响不到1%的肢端肥大症患者,典型肢端肥大症的误诊可能导致不适当的垂体手术。异位肢端肥大症有四种类型:1)中枢异位 GH 分泌:需要进行仔细的横断面成像以排除异位垂体腺瘤。2)外周性 GH 分泌:极为罕见。3) 中枢异位 GHRH 分泌:垂体手术后发现ellar神经节细胞瘤,免疫组化对GHRH呈阳性反应。4) 外周 GHRH 分泌:最常见的异位肢端肥大症是由外周分泌 GHRH 的 NETs 引起的。肿瘤较大,通常位于肺部或胰腺。慢性 GHRH 刺激导致的垂体增生很难被发现,或在核磁共振成像中被误诊为垂体腺瘤。测量血清 GHRH 水平是一种特异而有用的诊断工具。分泌GHRH的NET手术通常可以治愈。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The clinical and biochemical spectrum of ectopic acromegaly

Ectopic acromegaly is a rare condition caused by extrapituitary central or peripheral neuroendocrine tumours (NET) that hypersecrete GH or, more commonly, GHRH. It affects less than 1% of acromegaly patients and a misdiagnosis of classic acromegaly can lead to an inappropriate pituitary surgery. Four types of ectopic acromegaly have been described: 1) Central ectopic GH-secretion: Careful cross-sectional imaging is required to exclude ectopic pituitary adenomas. 2) Peripheral GH secretion: Extremely rare. 3) Central ectopic GHRH secretion: Sellar gangliocytomas immunohistochemically positive for GHRH are found after pituitary surgery. 4) Peripheral GHRH secretion: The most common type of ectopic acromegaly is due to peripheral GHRH-secreting NETs. Tumours are large and usually located in the lungs or pancreas. Pituitary hyperplasia resulting from chronic GHRH stimulation is difficult to detect or can be misinterpreted as pituitary adenoma in the MRI. Measurement of serum GHRH levels is a specific and useful diagnostic tool. Surgery of GHRH-secreting NETs is often curative.

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来源期刊
CiteScore
11.90
自引率
0.00%
发文量
77
审稿时长
6-12 weeks
期刊介绍: Best Practice & Research Clinical Endocrinology & Metabolism is a serial publication that integrates the latest original research findings into evidence-based review articles. These articles aim to address key clinical issues related to diagnosis, treatment, and patient management. Each issue adopts a problem-oriented approach, focusing on key questions and clearly outlining what is known while identifying areas for future research. Practical management strategies are described to facilitate application to individual patients. The series targets physicians in practice or training.
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