内分泌肿瘤的共同特征

A. Jouinot, Fidéline Bonnet-Serrano, J. Bertherat
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引用次数: 0

摘要

本章重点关注由内分泌腺引起的内分泌肿瘤,根据世界卫生组织2017年分类的定义:垂体、甲状腺、甲状旁腺、肾上腺皮质、肾上腺髓质和肾上腺外副神经节以及神经内分泌胰腺的肿瘤。这些肿瘤在病理生理学、诊断检查和临床管理方面具有特定的特征。遗传背景是常见的,包括对一种特定肿瘤类型的遗传易感性,如垂体腺瘤(AIP),原发性双侧大结节增生(ARMC5),或嗜铬细胞瘤/副神经节瘤(SDH基因),或多发性内分泌肿瘤(MEN1和RET)。激素检查,包括基础和动态检测,以及基于激素膜受体的放射学和核成像,是诊断过程的关键步骤,以评估激素分泌的数量和来源,以及肿瘤的扩展。内分泌肿瘤的治疗应结合肿瘤块的治疗(在大多数情况下主要是手术)和激素分泌的控制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Common Features of Endocrine Tumours
This chapter focuses on endocrine tumours arising from endocrine glands, as defined by the World Health Organization 2017 classification: tumours of the pituitary, the thyroid, the parathyroid, the adrenal cortex, the adrenal medulla, and extra-adrenal paraganglia, and the neuroendocrine pancreas. These tumours share specific features in terms of pathophysiology, diagnosis work-up, and clinical management. Genetic background is frequent and includes genetic predispositions to one specific tumour type, such as pituitary adenoma (AIP), primary bilateral macronodular hyperplasia (ARMC5), or pheochromocytoma/paraganglioma (SDH genes), or to multiple endocrine neoplasias (MEN1 and RET). Hormonal work-up, with basal and dynamic assays, and radiological as well as nuclear imaging based on hormone membrane receptors, are the key steps of the diagnosis process, to evaluate the amount and origin of hormonal secretion, as well as the tumour extension. Management of endocrine tumours should associate both the treatment of the tumour mass, in most cases primarily with surgery, and the control of hormone secretion.
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