BEST PRACTICE & RESEARCH CLINICAL ENDOCRINOLOGY & METABOLISM 是一本专题系列出版物，将最新原创研究的结果整合到实用的、基于证据的评论文章中，旨在解决诊断、治疗和患者管理等关键临床问题。每个问题都遵循一个问题- 面向方法，侧重于要解决的关键问题，明确定义已知内容并突出未来研究的主题。管理是用实际术语描述的，以便它可以应用于个体患者。该系列针对在实践中或培训中的医生。

Best Practice and Research: Clinical Endocrinology and Metabolism is a specialized series that integrates the latest original research findings into practical, evidence-based review articles aimed at addressing key clinical issues such as diagnosis, treatment, and patient management. Each issue follows a thematic approach, focusing on critical questions that need to be addressed, clearly defining known information, and highlighting future research directions. Management strategies are described in practical terms to facilitate application to individual patients. This series is targeted at physicians in practice or training.

《Best Practice and Research: Clinical Endocrinology and Metabolism》是一本专题系列出版物，旨在将最新的原创研究成果整合成实用的、基于证据的评论文章，解决诊断、治疗和患者管理等关键临床问题。每一期都围绕一个特定主题展开，重点关注需要解决的关键问题，明确已知信息，并突出未来研究的方向。管理策略以实际术语描述，以便能够应用于个体患者。该系列主要面向在临床实践或培训中的医生。

Best Practice and Research: Clinical Endocrinology and Metabolism

Precision medicine describes a target-related approach to tailoring diagnosis and treatment of the individual patient. While this personalized approach is revoluzionizing many areas of oncology, it is quite late in the field of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs), in which there are few molecular alterations to be therapeutically targeted. We critically reviewed the current evidence about precision medicine in GEP NENs, focusing on potential clinically relevant actionable targets for GEP NENs, such as the mTOR pathway, MGMT, hypoxia markers, RET, DLL-3, and some general agnostic targets. We analysed the main investigational approaches with solid and liquid biopsies. Furthermore, we reviewed a model of precision medicine more specific for NENs that is the theragnostic use of radionuclides. Overall, currently no true predictive factors for therapy have been validated so far in GEP NENs, and the personalized approach is based more on clinical thinking within a NEN-dedicated multidisciplinary team. However, there is a robust background to suppose that precision medicine, with the theragnostic model will yield new insights in this context soon.

Nicola Fazio

Anna La Salvia

Best Practice & Research Clinical Endocrinology & Metabolism

Precision medicine in gastroenteropancreatic neuroendocrine neoplasms: Where are we in 2023?

2023年胃肠胰神经内分泌肿瘤的精准医学：我们处于什么位置？

Endocrine disrupting chemicals (EDCs) are compounds that alter the structure and function of the endocrine system and may be contributing to disorders of the reproductive, metabolic, neuroendocrine and other complex systems. Typically, these outcomes cannot be modeled in cell-based or other simple systems necessitating the use of animal testing. Appropriate animal model selection is required to effectively recapitulate the human experience, including relevant dosing and windows of exposure, and ensure translational utility and reproducibility. While classical toxicology heavily relies on inbred rats and mice, and focuses on apical endpoints such as tumor formation or birth defects, EDC researchers have used a greater diversity of species to effectively model more subtle but significant outcomes such as changes in pubertal timing, mammary gland development, and social behaviors. Advances in genomics, neuroimaging and other tools are making a wider range of animal models more widely available to EDC researchers.

Heather B. Patisaul

Suzanne E. Fenton

David Aylor

Animal models of endocrine disruption

内分泌干扰的动物模型

Pheochromocytomas and paragangliomas (PPGLs) represent a unique subset of neuroendocrine neoplasms (NENs) characterized by their genetic diversity and potential for catecholamine secretion. Similar to epithelial NENs, all PPGLs are classified as malignant neoplasms that are associated with a variable risk of metastatic spread. PPGLs arise from neuroendocrine cells of the adrenal medulla (intra-adrenal paraganglia) or extra-adrenal paraganglia. Advances over the past two decades have significantly enhanced our understanding of the biological and genetic underpinnings of these neoplasms, resulting in robust genotype-phenotype (e.g., morphology, anatomic distribution, catecholamine profile, biomarker profile, risk of metastasis) correlations that guide diagnosis and prognostication. The 2022 WHO classification of PPGLs emphasizes a shift away from morphology-only diagnostic approaches by ensuring the integration of morphology with functional, structural and pathogenesis-related biomarker studies into routine pathology practice when assessing PPGLs. This paradigm is critical in distinguishing metastatic disease from multifocal primary tumors, particularly in patients with germline mutations - a hallmark of PPGLs, with germline susceptibility observed in at least 40 % of cases. This review provides practicing pathologists with a concise update on modern diagnostic and risk assessment strategies for PPGLs, focusing on the integration of biomarkers, genetic profiling, and morphological features. It also addresses emerging challenges, such as identifying metastatic potential and distinguishing these from synchronous lesions, to improve multidisciplinary care of these patients.

Ozgur Mete

C Christofer Juhlin

Best Pract Res Clin Endocrinol Metab

Recent progress in the pathologic classification of pheochromocytomas and paragangliomas

嗜铬细胞瘤和副神经节瘤病理分类的最新进展

Christina Pamporaki

Ruth T Casey

Current views on paediatric phaeochromocytoma and paraganglioma with a focus on newest guidelines

Xiao Guan

Minghao Li

Yingxian Pang

Yao He

Jing Wang

Xiaowen Xu

Kai Cheng

Zhi Li

Longfei Liu

Recent advances in algorithms predicting hemodynamic instability undergoing surgery for phaeochromocytoma and paraganglioma

预测嗜铬细胞瘤和副神经节瘤手术中血流动力学不稳定的算法最新进展

Immunotherapy represents a revolutionary advancement in cancer treatment, which has traditionally focused on T cells; however, the role of B cells in cancer immunotherapy has gained interest because of their role in antigen presentation, antibody production, and cytokine release. In this study, we examined the role of B cells in previously developed intratumoral MBTA therapy (mannan-BAM, TLR ligands, and anti-CD40 antibody) in murine models of MTT pheochromocytoma. The results indicated that B cells significantly enhance the success of MBTA therapy, with wild-type mice exhibiting a lower tumor incidence and smaller tumors compared with B cell-deficient mice. Increased IL-6 and TNF-alpha levels indicated severe inflammation and a potential cytokine storm in B cell-deficient mice. Neutralization of TNF-alpha ameliorated these complications but resulted in increased tumor recurrence. The results highlight the important role of B cells in enhancing the immune response and maintaining immune homeostasis during MBTA therapy. Our findings offer new insights into improving therapeutic outcomes.

Ondrej Uher

Katerina Hadrava Vanova

Katerina Petrlakova

Rachael Labitt

Radka Lencova

Andrea Frejlachova

Juan Ye

Herui Wang

Michal Masarik

Jan Zenka

Zhengping Zhuang

Karel Pacak

Role of B cells in intratumoral MBTA immunotherapy of murine pheochromocytoma model

小鼠嗜铬细胞瘤模型中B细胞在肿瘤内MBTA免疫治疗中的作用

Phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours with a strong genetic predisposition, involving over 20 genes and with germline pathogenic variants identified in 40 % of cases. The succinate dehydrogenase (SDHx) genes are the most commonly implicated in hereditary PPGLs, accounting for 20 % of cases, and present unique diagnostic and treatment challenges due to their potential for multiple, recurrent, and aggressive manifestations, often necessitating lifelong follow-up. Over the past two decades, advances in biochemical and imaging assessments, management, and follow-up protocols have significantly improved care for both adult and paediatric patients. These advances include next-generation sequencing, new biochemical tests, cluster-specific functional imaging, and improved surgical and radiotherapy techniques, such as stereotactic surgery and peptide receptor radionuclide therapy (PRRT). International consensus guidelines have been developed to standardise the management of patients with SDHx pathogenic variants, emphasising multidisciplinary approaches and frequent tumour board discussions. These guidelines, summarised below, cover recommendations for initial genetic testing, tumour screening, follow-up care, and management of patients and asymptomatic carriers.

Charlotte Lussey-Lepoutre

Ashley Grossman

David Taieb

Laurence Amar

Overview of recent guidelines and consensus statements on initial screening and management of phaeochromocytoma and paraganglioma in SDHx pathogenic variant carriers and patients

近期关于SDHx致病变异携带者和患者中嗜铬细胞瘤和副神经节瘤初始筛查与管理的指南和共识声明综述

Abbreviation	Best. Pract. Res. Clin. Endocrinol. Metab.
Journal Impact	6.05
Quartiles(Global)	ENDOCRINOLOGY & METABOLISM(Q1)

ISSN	1521-690X, 1532-1908
h-index	127

Publication Information	Publisher: Bailliere Tindall Ltd，Publishing cycle: Quarterly，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 1999，Proportion of original research papers: 80.00%，Self Citation Rate:0.00%， Gold OA Rate: 25.63%
Average review cycle	网友分享经验：较慢,6-12周
Average recruitment ratio	网友分享经验：一般

Best Practice and Research: Clinical Endocrinology and Metabolism

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Precision medicine in gastroenteropancreatic neuroendocrine neoplasms: Where are we in 2023?

Animal models of endocrine disruption

Recent progress in the pathologic classification of pheochromocytomas and paragangliomas

Current views on paediatric phaeochromocytoma and paraganglioma with a focus on newest guidelines

Recent advances in algorithms predicting hemodynamic instability undergoing surgery for phaeochromocytoma and paraganglioma

Recent progress in the pathologic classification of pheochromocytomas and paragangliomas

Current views on paediatric phaeochromocytoma and paraganglioma with a focus on newest guidelines

Recent advances in algorithms predicting hemodynamic instability undergoing surgery for phaeochromocytoma and paraganglioma

Role of B cells in intratumoral MBTA immunotherapy of murine pheochromocytoma model

Overview of recent guidelines and consensus statements on initial screening and management of phaeochromocytoma and paraganglioma in SDHx pathogenic variant carriers and patients