Table 12.

Individualized follow-up of patients with a history of a PPGL depending on the underlying mutation status and disease characteristics

Follow-up (history of a PPGL)	High risk	Intermediate risk	Low risk
	History of metastatic PPGL, history of sympathetic PGL, SDHA, SDHB, and FH (FH limited data), HIF2A/EPAS1	History of head and neck PGL, history of high-risk PCC (noradrenergic, ≥5 cm, recurrent, multiple) SDHAF2, SDHC, SDHD, VHL, NF1, MAX, TMEM127, RET with high/moderate risk for PCC	History of low-risk PCC (adrenergic, <5 cm), RET with low risk for PCC
Clinical, biochemistry	6-12 months (for HIF2A/EPAS1including hematocrit)	12 months (6 months for high-risk PCC)	12 months
Imaging (MRI base of the skull to pelvis/ MRI base of the skull, neck, abdomen, pelvis plus low-dose contrast-enhanced chest CT, alternating, for cluster 1; MRI abdomen/pelvis for cluster 2)	12-24 months (with history of disease initially 12, then 12-24 months) 6-12 months for history of very large primary PPGLs or those with large necrosis, high Ki67, and vascular and lymphatic invasion	24-36 months for SDHAF2, SDHC, SDHD (24 months for SDHD),VHL At least every 5 years for NF1, MAX, TMEM127, RET (only abdominal/pelvic MRI)	Optional
Special cases	For HIF2A/EPAS1: optic fundus examination every 12 months; PCC ≥5 cm: preoperative staging with additional contrast-enhanced chest CT or functional imaging History of nonfunctioning PPGL: Alternating, MRI (base of the skull to pelvis)/MRI base of the skull/neck/abdomen/pelvis plus low-dose contrast-enhanced chest CT every 24 months History of metastatic PPGL/ sympathetic PGL: functional imaging 3-6 months postsurgery, afterwards, alternating, yearly MRI (base of the skull to pelvis)/MRI base of the skull/neck/abdomen/pelvis plus low-dose chest CT, possibly functional imaging every 24-36 months	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; RET mutations: risk of primary hyperparathyroidism and medullary thyroid carcinoma (every 12 months calcitonin, calcium, PTH if applicable)
Postsurgery	Clinical and biochemical follow-up 3-6 weeks after surgery (after recovery)