First International Randomized Study in Malignant Progressive Pheochromocytoma and Paraganglioma

Inclusion Criteria: * Diagnosis of malignant PPGL, based on imaging or biopsy evidence of metastases in liver, bones, lungs and or lymph nodes, combined with at least one of two further confirmatory diagnoses: 1. diagnosis of PPGL from histopathological review of resected or biopsied tissue performed by a skilled pathologist (centralized review will be performed in all cases either before enrolment in case of any doubt or during the study); or 2. in patients where tumor tissue is unavailable for formal pathological review, from combined biochemical and functional imaging evidence of PPGL (e.g., MIBG scintigraphy combined with consistently and highly elevated plasma or urine levels of metanephrines). * Metastatic disease not amenable to surgical resection * Pre-treated or not * Whatever the genetic status (sporadic or inherited) * Evaluable disease according to RECIST 1.1 criteria * Progressing disease within 18 months at imaging prior to randomization according to RECIST. The recent scan indicating progression may be used as the screening scan if within 28 days of randomization * ECOG performance status 0-2 * Life expectancy ≥ 6 months as prognosticated by the physician * Age ≥18 years, no superior limit * Adequate bone marrow reserve (Hb \> 8, neutrophils ≥ 1500/mm³ and platelets ≥80.000/mm³) * Effective contraception in pre-menopausal female and male patients * Negative pregnancy test * Patient´s signed written informed consent * Ability to comply with the protocol procedu…