Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy.
Clinical Trial Center, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
Tumori. 2022 Dec;108(6):526-540. doi: 10.1177/03008916221078621. Epub 2022 May 20.
Pheochromocytomas and paragangliomas (PPGLs) can metastasize in approximately 15-20% of cases. This review discusses the available evidence on the biology and treatment of metastatic PPGLs. Chemotherapy is the first-line treatment option for this evolving and symptomatic disease. In patients with high MIBG uptake and positive PETGa-68, radiometabolic treatment may be considered. The efficacy of sunitinib has been shown in observational studies, and pembrolizumab has been evaluated in phase II clinical studies, while other agents investigated in this setting are anti-angiogenic drugs cabozantinib, dovitinib, axitinib and lenvatinib. As these agents' efficacy and safety data, alone or in combination, are scant and based on few treated patients, enrollment in clinical trials is mandatory. Future therapeutic options may be represented by DNA repair system inhibitors (such as olaparib), HIF2 inhibitors and immunotherapy.
嗜铬细胞瘤和副神经节瘤（PPGLs）在大约 15-20%的病例中可能发生转移。这篇综述讨论了转移性 PPGLs 的生物学和治疗方面的现有证据。化疗是这种不断发展和有症状的疾病的一线治疗选择。对于 MIBG 摄取高且 PETGa-68 阳性的患者，可以考虑放射性代谢治疗。在观察性研究中已经证明了舒尼替尼的疗效，而 pembrolizumab 已在 II 期临床试验中进行了评估，而在该治疗环境中研究的其他药物是抗血管生成药物卡博替尼、多韦替尼、阿昔替尼和仑伐替尼。由于这些药物的疗效和安全性数据，单独或联合使用，数据稀少且基于少数治疗患者，因此必须参加临床试验。未来的治疗选择可能是 DNA 修复系统抑制剂（如奥拉帕利）、HIF2 抑制剂和免疫疗法。
