Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors arising from chromaffin cells...33 patients were diagnosed with metastatic PPGLs and received systemic treatment....In CVD treatment, patients with SDHB/SDHD mutation (either germline or somatic) were significantly associated with superior OS & PFS compared with the non-SDHB/SDHD mutation group, with a median OS of 94.0 months vs. 5.7 months (P = 0.01) and a median PFS of 20.1 months vs. 1.6 months (P = 0.03), respectively. In the SDHB/SDHD mutation group, the ORR was 50% (3/6, all PR), and the DCR was 100% (n=6), but in a non-SDHB/SDHD mutation group, the ORR was 11% (1/9, PR) and the DCR was 44% (n=4).