Pheochromocytomas are rare neuroendocrine chromaffin-derived tumors that arise within the adrenal medulla. They are usually benign, but if not diagnosed or if left untreated, they can have devastating consequences. Clinical consideration of the diagnosis is paramount, as they may have protean manifestations, and a high index of suspicion is essential if serious consequences are to be avoided.

An accurate biochemical diagnosis is crucial for the management of these patients: either plasma or urinary metanephrines are both highly sensitive and specific if correctly employed, but knowledge of pre- and post-analytic interference is essential.

Diagnostic imaging with cross-sectional CT and/or MRI offers high sensitivity in their detection, but lack specificity. The introduction of PET/CT/MR has led to a dramatic improvement in the localization of both pheochromocytomas and paragangliomas, together with the increasing availability of new functional imaging radionuclides. Optimal investigation and accurate diagnosis is best achieved at ‘centers of excellence’ with expert multidisciplinary teams.

嗜铬细胞瘤是在肾上腺髓质内出现的罕见的神经内分泌嗜铬细胞源性肿瘤。它们通常是良性的，但如果不被诊断或不加以治疗，可能会造成毁灭性后果。诊断的临床考虑是至关重要的，因为它们可能具有蛋白质的表现，如果要避免严重的后果，高度怀疑是必不可少的。

准确的生化诊断对于这些患者的治疗至关重要：如果正确使用血浆或尿中的肾上腺素，既敏感又特异性高，但是了解分析前后的干扰至关重要。

使用断层CT和/或MRI进行诊断成像可在检测中提供高灵敏度，但缺乏特异性。PET / CT / MR的引入极大地改善了嗜铬细胞瘤和副神经节瘤的定位，并增加了新功能成像放射性核素的可用性。在专业的多学科团队的“卓越中心”，最佳的调查和准确的诊断是最好的。