Table 5.

Follow-up of cluster 1A/1B mutation carriers with a history of a PPGL

Follow-up of cluster 1 mutation carriers with a history of a PPGL	History of metastatic PPGL, history of sympathetic PGL, SDHA/B, FH HIF2A/EPAS1-related PPGLs	History of head and neck PGL, SDHC/D/AF2, VHL
Biochemistry	6-12 months (for HIF2A/EPAS1 including hematocrit)	12 months
Imaging (MRI base of the skull to pelvis, possibly alternating with low-dose chest CT plus MRI base of the skull, neck, abdomen, pelvis)	12-24 months (initially 12, then 12-24 months)	24-36 months (24 months for SDHD) VHL mutations: risk of renal cell cancer, consider abdominal MRI every 12 months; optic fundus examination every 12 months; CNS tumors, CNS MRI every 24-36 months.

Abbreviations: CNS, central nervous system; CT, computed tomography; MRI, magnetic resonance imaging; PGL, paraganglioma; PPGL, pheochromocytoma/paraganglioma.