Abdominal paragangliomas and pheochromocytomas (PPGLs) are rare neuroendocrine tumors of the infradiaphragmatic paraganglia and adrenal medulla, respectively. Although few pathologists outside of endocrine tertiary centers will ever diagnose such a lesion, the tumors are well known through the medical community—possible due to a combination of the sheer rarity, their often-spectacular presentation due to excess catecholamine secretion as well as their unrivaled coupling to constitutional susceptibility gene mutations and hereditary syndromes. All PPGLs are thought to harbor malignant potential, and therefore pose several challenges to the practicing pathologist. Specifically, a responsible diagnostician should recognize both the capacity and limitations of histological, immunohistochemical, and molecular algorithms to pinpoint high risk for future metastatic disease. This focused review aims to provide the surgical pathologist with a condensed update regarding the current strategies available in order to deliver an accurate prognostication of these enigmatic lesions.

腹部副神经节瘤和嗜铬细胞瘤（PPGLs）分别是膈下副神经节和肾上腺髓质的罕见神经内分泌肿瘤。尽管内分泌三级中心以外的病理学家很少会诊断出这样的病变，但这种肿瘤在医学界是众所周知的——这可能是由于其极为罕见、由于儿茶酚胺分泌过多而引起的经常壮观的表现以及无与伦比的与体质易感基因突变和遗传综合征的耦合。所有 PPGL 都被认为具有恶性潜能，因此对执业病理学家提出了一些挑战。具体而言，负责任的诊断师应认识到组织学、免疫组织化学、和分子算法来确定未来转移性疾病的高风险。本综述旨在为外科病理学家提供有关当前可用策略的简要更新，以便对这些神秘病变进行准确的预测。