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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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	Sensitivity	Specificity	Comments, Study
Metanephrine and catecholamine levels, URINE	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	99%	89%	Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030
Urine Catecholamines	86%	88%	Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Specific Findings

Urine Vanillylmandelic Acid

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

77%

93%

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

Sensitive Findings

Urinary Fractionated Metanephrines

97%

45%

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

Poorly Diagnostic Findings

Plasma Catecholamines

84%

81%

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

Findings With Unspecified Accuracy

Finding	Sensitivity	Specificity	Comments, Study
Clonidine suppression test	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	No accuracy specified.		Elevated in other neuroendocrine tumors as well Study: no study specified.
Neuropeptide Y, Elevated	No accuracy specified.		Study: no study specified.
MIBG scintigraphy	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	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	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	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	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.