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Pheochromocytoma: Sensitivity and Specificity

Introduction: Can be sporatic or hereditary (von Hippel-Lindau, MEN 2, and neurofibromatosis 1). Note that the sensitivities and specificities of the catecholamine tests differ between the two groups (JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030).

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Tags: Endocrine Neoplasm Tag this Diagnosis.

The sensitivity and specificity of findings for Pheochromocytoma are listed below. See the left navigation bar to change the display.

Sensitive and Specific Findings

Finding SensitivitySpecificity Comments, Study
Metanephrine and catecholamine levels, URINE Edit 98%98%

Measurement of fractionated metanephrines and catecholamines in 24-hour urine collection

Note: TCA's and other psychoactive drugs may interfere with test results

Note: Won't be positive for a dopamine excreting tumor

Study: A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines.

Sawka AM; Jaeschke R; Singh RJ; Young WF Jr

J Clin Endocrinol Metab 2003 Feb;88(2):553-8.

Clinical review 164: The laboratory diagnosis of adrenal pheochromocytoma: the Mayo Clinic experience.

Kudva YC; Sawka AM; Young WF Jr

J Clin Endocrinol Metab 2003 Oct;88(10):4533-9.

The diagnostic efficacy of urinary fractionated metanephrines measured by tandem mass spectrometry in detection of pheochromocytoma.

Perry CG; Sawka AM; Singh R; Thabane L; Bajnarek J; Young WF Jr

Clin Endocrinol (Oxf). 2007 May;66(5):703-8. Epub 2007 Mar 27.

Plasma Free Metanephrines Edit 99%89%

Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Urine Catecholamines Edit 86%88%

Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Specific Findings

Finding SensitivitySpecificity Comments, Study
Urine Vanillylmandelic Acid Edit 64%95%

elevated

Study: Biochemical diagnosis of pheochromocytoma: which test is best?

Lenders JW; Pacak K; Walther MM; Linehan WM; Mannelli M; Friberg P; Keiser HR; Goldstein DS; Eisenhofer G

JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Urine Total Metanephrines Edit 77%93%

Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Sensitive Findings

Finding SensitivitySpecificity Comments, Study
Urinary Fractionated Metanephrines Edit 97%45%

Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Poorly Diagnostic Findings

Finding SensitivitySpecificity Comments, Study
Plasma Catecholamines Edit 84%81%

Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Findings With Unspecified Accuracy

Finding SensitivitySpecificity Comments, Study
Clonidine suppression test Edit No accuracy specified.

This is test is used to confirm a positive result of increased catecholamines in plasma or urine.

The clonidine suppression test is used to distinguish between pheochromocytoma and false-positive increases in plasma catecholamines and fractionated metanephrines.

Clonidine suppresses the release of catecholamines from neurons but does not affect the catecholamine secretion from a pheochromocytoma. (UpToDate)

Study: The clonidine suppression test for pheochromocytoma. A review of its utility and pitfalls.

Sjoberg RJ; Simcic KJ; Kidd GS

Arch Intern Med 1992 Jun;152(6):1193-7.

Chromogranin A, Elevated Edit No accuracy specified.

Elevated in other neuroendocrine tumors as well

Study: no study specified.

Neuropeptide Y, Elevated Edit No accuracy specified.

Study: no study specified.

MIBG scintigraphy Edit No accuracy specified.

123-I-metaiodobenzylguanidine (MIBG) scintigraphy can detect very small tumors that may be missed on CT or MRI.

Study: Pheochromocytoma: new concepts and future trends.

Bravo EL

Kidney Int 1991 Sep;40(3):544-56.

Headache Edit No accuracy specified.

Part of classic triad of headache, tachycardia and sweating

Study: A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience.

Stein PP; Black HR

Medicine (Baltimore) 1991 Jan;70(1):46-66.

Tachycardia Edit No accuracy specified.

Part of triad of headache, tachycardia and sweating

Study: A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience.

Stein PP; Black HR

Medicine (Baltimore) 1991 Jan;70(1):46-66.

Sweating Edit No accuracy specified.

Part of the triad of headache, tachycardia and sweating

Study: A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience.

Stein PP; Black HR

Medicine (Baltimore) 1991 Jan;70(1):46-66.

Hypertension, paroxysmal Edit No accuracy specified.

Seen in about half of people with pheochromocytoma

Study: Circumstances of discovery of phaeochromocytoma: a retrospective study of 41 consecutive patients.

Baguet JP; Hammer L; Mazzuco TL; Chabre O; Mallion JM; Sturm N; Chaffanjon P

Eur J Endocrinol 2004 May;150(5):681-6.