Metastatic pheochromocytomas and paragangliomas (MPPGs) are rare neuroendocrine tumors. Most patients present with advanced disease that is associated with manifestations of catecholamine release. Surgical resection of the primary tumor and ablative therapies of metastases—whenever possible—may improve clinical outcomes and, perhaps, lengthen the patient's overall survival. Significant steps in understanding the genetic alterations linked to MPPGs and scientific progress made on cancers that share a similar pathogenesis are leading to the recognition of potential systemic therapeutic options. Data derived from clinical trials evaluating targeted therapies such as tyrosine kinase inhibitors, radiopharmaceuticals, immunotherapy, and combinations of these will likely improve the outcomes of patients with advanced and progressive MPPGs. Exemplary of this success is the recent approval in the United States of the high-specific-activity iodine¹³¹ meta-iodine-benzylguanidine (MIBG) for patients with unresectable and progressive MPPGs that express the noradrenaline transporter. This review will discuss the therapeutic approaches for patients with MPPGs.

转移性嗜铬细胞瘤和神经节旁瘤（MPPG）是罕见的神经内分泌肿瘤。大多数患者表现出与儿茶酚胺释放相关的晚期疾病。如果可能，手术切除原发肿瘤和消融转移疗法可能会改善临床效果，并可能延长患者的整体生存期。理解与MPPG相关的遗传变异的重要步骤以及在具有相似发病机理的癌症方面取得的科学进展，正导致人们认识到潜在的全身性治疗选择。来自评估靶向疗法（例如酪氨酸激酶抑制剂，放射性药物，免疫疗法以及这些疗法的组合）的临床试验的数据可能会改善晚期和进行性MPPG患者的预后。¹³¹间碘苄基胍（MIBG）用于表达去甲肾上腺素转运蛋白的不可切除和进行性MPPG的患者。本文将讨论MPPG患者的治疗方法。