Pheochromocytomas and paragangliomas (PPGLs) generally grouped together are rare catecholamine-secreting endocrine tumors. Symptoms of catecholamine excess are non-specific and therefore a high index of suspicion in children with sustained hypertension, family history of endocrine tumors, or features of syndromes associated with PPGLs leads to a timely diagnosis and treatment. Free metanephrines in the plasma or 24-h urine are the preferred tests to establish catecholamine excess. Considerations for false-positive conditions improve diagnostic yield and accuracy. Functional imaging, targeting either specific cell membrane transporters or vesicular catecholamine transport systems, is indicated for incidental lesions suspicious for PPGLs with inconclusive biochemical testing, assessment of regional extension or multifocality, and exclusion of metastases. Surgery is the mainstay of treatment for PPGLs. Preoperatively, sequential use of alpha adrenergic receptor blockade and volume expansion followed by beta blockade is mandatory to reduce intraoperative intravascular instability and blood pressure fluctuation due to tumor manipulation. Since genetic mutations have been reported in tumor susceptibility genes in nearly 50% of patients with PPGLs, genetic counselling and testing should be considered in all patients with a confirmed tumor.

中文翻译：

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嗜铬细胞瘤和副神经节瘤-诊断，评估和管理的最新进展。

嗜铬细胞瘤和副神经节瘤（PPGL）通常分为几类，是分泌儿茶酚胺的罕见内分泌肿瘤。儿茶酚胺过量的症状是非特异性的，因此对于患有持续性高血压，内分泌肿瘤的家族病史或与PPGLs相关的综合征特征的儿童高度怀疑，可及时诊断和治疗。血浆或24小时尿液中的游离偏肾上腺素是确定儿茶酚胺过量的首选试验。假阳性条件的考虑因素可提高诊断率和准确性。针对功能不确定的生化测试，区域扩展或多焦点评估，针对特定细胞膜转运蛋白或囊泡儿茶酚胺转运系统的功能性影像学可用于PPGL可疑的偶然病变，和排除转移。外科手术是PPGL的主要治疗手段。术前，必须先后顺序使用α肾上腺素受体阻滞剂和扩大容量，然后再进行β阻滞剂，以减少术中血管内的不稳定性和由于肿瘤操作引起的血压波动。由于已经报道了近50％的PPGL患者的肿瘤易感基因中存在基因突变，因此在所有确诊肿瘤的患者中都应考虑进行遗传咨询和检测。