A molecular biology–based taxonomy has been proposed for pheochromocytoma and paraganglioma (PPGL). Data from the Cancer Genome Atlas revealed clinically relevant prognostic and predictive biomarkers and stratified PPGLs into three main clusters. Each subgroup has a distinct molecular–biochemical–imaging signature. Concurrently, new methods for biochemical analysis, functional imaging, and medical therapies have also become available. The research community now strives to match the cluster biomarkers with the best intervention. The concept of precision medicine has been long awaited and holds great promise for improved care. Here, we review the current and future PPGL classifications, with a focus on hereditary syndromes. We discuss the current strengths and shortcomings of precision medicine and suggest a condensed manual for diagnosis and treatment of both adult and pediatric patients with PPGL. Finally, we consider the future direction of this field, with a particular focus on how advanced molecular characterization of PPGL can improve a patient’s outcome, including cures and, ultimately, disease prevention.

中文翻译：

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嗜铬细胞瘤和副神经节瘤的新观点：分子分类

已经提出了一种基于分子生物学的分类方法用于嗜铬细胞瘤和副神经节瘤（PPGL）。癌症基因组图谱的数据显示了临床相关的预后和预测性生物标志物，并将PPGLs分为三个主要类别。每个亚组都有独特的分子-生化-成像特征。同时，用于生化分析，功能成像和医学疗法的新方法也已可用。现在，研究界努力使集群生物标记物与最佳干预措施相匹配。精确医学的概念一直期待已久，并有望改善护理水平。在这里，我们回顾当前和未来的PPGL分类，重点关注遗传综合征。我们讨论了精密医学的当前优缺点，并提出了用于成人和小儿PPGL的诊断和治疗的简明手册。最后，我们考虑该领域的未来方向，特别关注PPGL的先进分子表征如何改善患者的预后，包括治愈方法以及最终预防疾病。