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1. Diagnostic tests for pheochromocytoma include the following:
2. Test selection criteria include the following:
3. Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. Studies are as follows:
4. Additional studies to rule out a familial syndrome in patients with confirmed pheochromocytoma include the following:

PET scanning: A promising technique for detection and localization of pheochromocytomas

Abdominal CT scanning: Has accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater

Genetic testing for mutations causing the MEN 2A and 2B syndromes

Scintigraphy: Reserved for biochemically confirmed cases in which CT scanning or MRI does not show a tumor

MRI: Preferred over CT scanning in children and pregnant or lactating women; has reported sensitivity of up to 100% in detecting adrenal pheochromocytomas

Consultation with an ophthalmologist to rule out retinal angiomas (VHL disease)

Serum intact parathyroid hormone level and a simultaneous serum calcium level to rule out primary hyperparathyroidism (which occurs in MEN 2A)

Use 24-hour urinary collection for catecholamines and metanephrines in patients at lower risk

Plasma metanephrine testing: 96% sensitivity, 85% specificity

24-hour urinary collection for catecholamines and metanephrines: 87.5% sensitivity, 99.7% specificity

Use plasma metanephrine testing in patients at high risk (ie, those with predisposing genetic syndromes or a family or personal history of pheochromocytoma)

Screening for mutations in the ret proto-oncogene (which give rise to MEN 2A and 2B)