Metastatic pheochromocytomas and paragangliomas: where are we?
Natalie PrinziFrancesca CortiMartina TorchioMonica NigerMaria AntistaFilippo PaganiTeresa BeninatoIolanda PuliceRoberta Elisa RossiJorgelina CoppaTommaso CascellaLuca GiacomelliMaria Di BartolomeoMassimo MilioneFilippo de BraudSara PuscedduPublished in: Tumori (2022)
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.
Keyphrases
- clinical trial
- phase ii
- dna repair
- squamous cell carcinoma
- small cell lung cancer
- end stage renal disease
- dna damage
- metastatic renal cell carcinoma
- newly diagnosed
- open label
- ejection fraction
- electronic health record
- randomized controlled trial
- endothelial cells
- mass spectrometry
- health insurance
- phase iii
- machine learning
- patient reported outcomes
- deep learning
- tyrosine kinase
- atomic force microscopy