嗜铬细胞瘤国际研讨会:致力于医疗保健专业人员和研究人员争取更好的患者结果的事件。

IF 4.1 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Endocrine-related cancer Pub Date : 2023-05-01 DOI:10.1530/ERC-23-0030
Karel Pacak, Roderick J Clifton-Bligh
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引用次数: 0

摘要

嗜铬细胞瘤和副神经节瘤(PPGLs)被定义为产生儿茶酚胺的神经内分泌肿瘤。最近在其管理、定位、治疗和监测方面的许多进展显著改善了PPGLs患者或与这些肿瘤发展相关的致病基因变异携带者的预后。目前,这些进展主要包括PPGL的分子分层分为7类,2017年WHO修订了这些肿瘤的定义,存在指向PPGL的特定临床特征,使用具有特定参考限值的血浆肾上腺素和3-甲氧基酪胺来评估PPGL的可能性(例如高危和低危患者),包括年龄特异性参考限值。核医学指南概述了聚类和转移性疾病特异性功能成像(主要是正电子发射断层扫描和间氧苄基胍显像)在PPGLs精确诊断定位中的应用,转移性疾病患者使用放化疗的指南,以及对无症状种系SDHx致病变异携带者的初始筛查和随访的国际共识。此外,新的合作努力,特别是基于多机构和全球倡议,现在被认为是提高我们对这些肿瘤和未来成功治疗甚至预防性干预的理解和知识的关键力量。
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International symposium on pheochromocytoma: an event of dedicated healthcare professionals and researchers striving for better patient outcomes.

Pheochromocytomas and paragangliomas (PPGLs) are defined as neuroendocrine tumors that produce catecholamines. Many recent advances in their management, localization, treatment, as well as surveillance have significantly improved outcomes for patients with PPGLs or carriers of pathogenic genetic variants linked to the development of these tumors. At present, those advances mainly include the molecular stratification of PPGLs into seven clusters, the 2017 WHO revised definition of these tumors, the presence of specific clinical features pointing toward PPGL, the use of plasma metanephrines and 3-methoxytyramine with specific reference limits to assess the likelihood of having a PPGL (e.g. patients at high and low risk) including age-specific reference limits, nuclear medicine guidelines outlining cluster- and metastatic disease-specific functional (here mainly positron emission tomography and metaiodobenzylguanidine scintigraphy) imaging in the precise diagnostic localization of PPGLs, the guidelines for using radio- vs chemotherapy for patients with metastatic disease, and the international consensus on initial screening and follow-up of asymptomatic germline SDHx pathogenic variant carriers. Furthermore, new collaborative efforts particularly based on multi-institutional and worldwide initiatives are now considered key forces in improving our understanding and knowledge about these tumors and future successful treatments or even preventative interventions.

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来源期刊
Endocrine-related cancer
Endocrine-related cancer 医学-内分泌学与代谢
CiteScore
7.80
自引率
2.60%
发文量
138
审稿时长
6-12 weeks
期刊介绍: Endocrine-Related Cancer is an official flagship journal of the Society for Endocrinology and is endorsed by the European Society of Endocrinology, the United Kingdom and Ireland Neuroendocrine Society, and the Japanese Hormones and Cancer Society. Endocrine-Related Cancer provides a unique international forum for the publication of high quality original articles describing novel, cutting edge basic laboratory, translational and clinical investigations of human health and disease focusing on endocrine neoplasias and hormone-dependent cancers; and for the publication of authoritative review articles in these topics. Endocrine neoplasias include adrenal cortex, breast, multiple endocrine neoplasia, neuroendocrine tumours, ovary, prostate, paraganglioma, parathyroid, pheochromocytoma pituitary, testes, thyroid and hormone-dependent cancers. Neoplasias affecting metabolism and energy production such as bladder, bone, kidney, lung, and head and neck, are also considered.
期刊最新文献
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