Management of Suspected Pheochromocytoma/Paraganglioma

Pre-Test Preparation and Collection

The American College of Cardiology recommends that plasma free metanephrines should ideally be collected from an indwelling venous catheter after the patient has been lying supine for 30 minutes to minimize false positive results 1, 2
Clinicians may bypass this ideal approach in routine screening, but marginally elevated results must prompt repetition under standardized conditions, as suggested by Clinical Cancer Research 3, 1
For high-risk populations, such as those with hereditary syndromes or strong clinical suspicion, use the indwelling catheter protocol from the outset 1

Results consistent with pheochromocytoma/paraganglioma, with levels ≥4 times the upper limit of normal, warrant immediate imaging to localize the lesion, according to Clinical Cancer Research and Praxis Medical Insights 3, 1, 4, 2
This degree of elevation is diagnostic regardless of collection method, with a high strength of evidence 1

The American Heart Association prefers MRI of abdomen and pelvis over CT due to risk of hypertensive crisis with IV contrast, as recommended by Praxis Medical Insights 1, 4, 2
If initial imaging is negative but biochemical evidence is positive, extend imaging to include chest and neck, as suggested by Praxis Medical Insights 1

Never perform fine needle biopsy of suspected pheochromocytoma before biochemical exclusion, as this can precipitate fatal hypertensive crisis, according to Praxis Medical Insights 1
Never initiate beta-blockade alone before alpha-blockade in suspected pheochromocytoma, as this can precipitate severe hypertensive crisis due to unopposed alpha-adrenergic stimulation, as recommended by Praxis Medical Insights 1, 2

Approximately 25-33% of patients with pheochromocytoma have germline mutations, according to Praxis Medical Insights 4, 2
Consider genetic testing in patients with family history, young age at diagnosis, bilateral or multifocal disease, or extra-adrenal location, as suggested by Praxis Medical Insights 1, 4, 2
SDHB mutations are associated with higher risk of aggressive behavior and metastatic disease, requiring more intensive surveillance, according to Praxis Medical Insights 1, 4

For von Hippel-Lindau syndrome, begin surveillance with plasma free metanephrines at age 2 years, as recommended by Clinical Cancer Research and Praxis Medical Insights 3, 1
For hereditary paraganglioma/pheochromocytoma syndromes, begin surveillance at age 6-8 years, according to Praxis Medical Insights 1

Repeat biochemical testing 14 days post-surgery to confirm complete resection, as suggested by Praxis Medical Insights 1
Follow-up every 3-4 months for 2-3 years, then every 6 months, with long-term surveillance due to risk of malignant recurrence, particularly those with SDHB mutations, extra-adrenal tumors, or tumors >5 cm, according to Praxis Medical Insights 1