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.

Pre-Test Probability (Prevalence): %.	Post-Test Probability (Predictive Value): %.	Switch to display mode.
Note that calculating probabilities from more than one** finding is inherently inaccurate because findings are not independent. (For example, using two positive findings that share a common pathogenesis is likely to overestimate the true probability.)

Sensitive and Specific Findings
Finding	Result	Sensitivity	Specificity
[ + ] Metanephrine and catecholamine levels, URINE	+ 0 -	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	+ 0 -	99%	89%
Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030
[ + ] Urine Catecholamines	+ 0 -	86%	88%
Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Specific Findings
Finding	Result	Sensitivity	Specificity
[ + ] Urine Vanillylmandelic Acid	+ 0 -	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	+ 0 -	77%	93%
Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Sensitive Findings
Finding	Result	Sensitivity	Specificity
[ + ] Urinary Fractionated Metanephrines	+ 0 -	97%	45%
Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Poorly Diagnostic Findings
Finding	Result	Sensitivity	Specificity
[ + ] Plasma Catecholamines	+ 0 -	84%	81%
Study: JAMA 2002 Mar 20;287(11):1427-34. PMID: 11903030

Findings With Unspecified Accuracy
Finding	Result	Sensitivity	Specificity
[ + ] 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.