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 Practice & Research Clinical Endocrinology & Metabolism

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。

Category	Quartile
医学	1
医学, 内分泌学与代谢	1

Recent progress in the pathologic classification of pheochromocytomas and paragangliomas

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