Division of Endocrinology and Metabolism, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Curr Opin Endocrinol Diabetes Obes. 2019 Jun;26(3):146-154. doi: 10.1097/MED.0000000000000476.
Metastatic pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors with variable prognosis. This review highlights recent studies on outcomes and management of patients with metastatic PPGL.
Latest advances were made in identifying predictors of favorable outcomes of patients with metastatic PPGL. Recent studies evaluated the efficacy of tyrosine kinase inhibitors, high-specific-activity radiopharmaceuticals, and peptide receptors radionuclide therapy in treatment of metastatic disease. Moreover, ongoing studies are assessing the effects of hypoxia-inducible factor 2αα and heat shock protein 90 inhibitors as potential therapies.
Several active studies are evaluating the efficacy of systemic chemo, immuno, radiopharmaceutical, and peptide receptor radionuclide therapies to relieve local and adrenergic symptoms and provide survival benefit for patients with symptomatic and/or progressive advanced metastatic PPGL. Owing to rarity and wide-outcome variability, multidisciplinary team effort and personalized approach are central in caring for patients with metastatic PPGL.
转移性嗜铬细胞瘤和副神经节瘤（PPGL）是罕见的神经内分泌肿瘤，具有不同的预后。本文重点介绍了转移性 PPGL 患者结局和管理的最新研究进展。
目前已经明确了预测转移性 PPGL 患者良好结局的因素。近期研究评估了酪氨酸激酶抑制剂、高比活度放射性药物和肽受体放射性核素疗法治疗转移性疾病的疗效。此外，正在进行的研究评估了缺氧诱导因子 2αα 和热休克蛋白 90 抑制剂作为潜在治疗方法的效果。
目前正在开展多项研究评估全身化疗、免疫、放射性药物和肽受体放射性核素疗法的疗效，以缓解局部和肾上腺素能症状，并为有症状和/或进展性晚期转移性 PPGL 患者提供生存获益。由于其罕见性和广泛的结局变异性，多学科团队的努力和个体化方法是治疗转移性 PPGL 患者的核心。
