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HelpMayo Test ID VH Vanillylmandelic Acid and Homovanillic Acid, Random, Urine
Ordering Guidance
In the past, this test has been used to screen for pheochromocytoma. However, vanillylmandelic acid (VMA) is not the analyte of choice to rule out a diagnosis of pheochromocytoma. Recommended tests for that purpose include:
-PMET / Metanephrines, Fractionated, Free, Plasma
-METAF / Metanephrines, Fractionated, 24 Hour, Urine
-CATU / Catecholamine Fractionation, Free, 24 Hour, Urine
Necessary Information
1. Patient's age is required.
2. All patients receiving L-dopa should be identified to the laboratory when this test is ordered.
3. Bactrim may interfere with detection of the analyte. All patients taking Bactrim should be identified to the laboratory when this test is ordered.
Specimen Required
Patient Preparation: Administration of L-dopa may falsely increase homovanillic acid and vanillylmandelic acid results; it should be discontinued 24 hours prior to specimen collection.
Supplies: Urine Tubes, 10 mL (T068)
Collection Container/Tube: Clean, plastic urine collection container
Submission Container/Tube: Plastic, 10-mL urine tube
Specimen Volume: 5 mL
Collection Instructions:
1. Collect a random urine specimen.
2. Adjust the urine pH to a level between 1 and 5 by adding 50% acetic acid or hydrochloric acid dropwise and checking the pH.
Useful For
Preferred first test for screening for catecholamine-secreting tumors in a random urine specimen when requesting both homovanillic acid and vanillylmandelic acid
Supporting a diagnosis of neuroblastoma
Monitoring patients with a treated neuroblastoma
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Reporting Name
VMA and HVA, Random, USpecimen Type
UrineSpecimen Minimum Volume
2 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Urine | Refrigerated (preferred) | 28 days | |
| Frozen | 180 days |
Reject Due To
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.Clinical Information
Elevated values of homovanillic acid (HVA), vanillylmandelic acid (VMA), and other catecholamine metabolites (eg, dopamine) may be suggestive of the presence of a catecholamine-secreting tumor (eg, neuroblastoma, pheochromocytoma, or other neural crest tumors). HVA and VMA levels may also be useful in monitoring patients who have been treated as a result of the above-mentioned tumors. HVA levels may also be altered in disorders of catecholamine metabolism: monamine oxidase-A deficiency can cause decreased urinary HVA values, while a deficiency of dopamine beta-hydrolase (the enzyme that converts dopamine to norepinephrine) can cause elevated urinary HVA values.
Reference Values
VANILLYLMANDELIC ACID
<1 year: <25.0 mg/g creatinine
1 year: <22.5 mg/g creatinine
2-4 years: <16.0 mg/g creatinine
5-9 years: <12.0 mg/g creatinine
10-14 years: <8.0 mg/g creatinine
≥15 years: <7.0 mg/g creatinine
HOMOVANILIC ACID
<1 year: <35.0 mg/g creatinine
1 year: <30.0 mg/g creatinine
2-4 years: <25.0 mg/g creatinine
5-9 years: <15.0 mg/g creatinine
10-14 years: <9.0 mg/g creatinine
≥15 years: <8.0 mg/g creatinine
Interpretation
Homovanillic acid (HVA) and vanillylmandelic acid (VMA) concentrations are elevated in more than 90% of patients with neuroblastoma; both tests should be performed. A positive test could be due to a genetic or nongenetic condition. Additional confirmatory testing is required.
A normal result does not exclude the presence of a catecholamine-secreting tumor.
Elevated HVA and VMA values are suggestive of a pheochromocytoma, but they are not diagnostic.
Cautions
No significant cautionary statements
Clinical Reference
1. Eisenhofer G: Monoamine-producing tumors. In: Rifai N, Chiu RWK, Young I, Burnham CD, Wittwer CT, eds. Tietz Textbook of Laboratory Medicine. 7th ed. Elsevier; 2023:765
2. Ormazabal A, Molero-Luis M, Garcia-Cazorla A, Artuch R. Biomarkers for the study of catecholamine and serotonin genetic diseases. In: Garg U, Smith LD, eds. Biomarkers in Inborn Errors of Metabolism: Clinical Aspects and Laboratory Determination. Elsevier; 2017:301-329
3. Strenger V, Kerbl R, Dornbusch HJ, et al. Diagnostic and prognostic impact of urinary catecholamines in neuroblastoma patients. Pediatr Blood Cancer. 2007;48(5):504-509
4. Barco S, Gennai I, Reggiardo G, et al. Urinary homovanillic and vanillylmandelic acid in the diagnosis of neuroblastoma: report from the Italian Cooperative Group for Neuroblastoma. Clin Biochem. 2014;47(9):848-852
5. Matthay KK, Maris JM, Schleiermacher G, et al. Neuroblastoma. Nat Rev Dis Primers. 2016;2:16078. doi:10.1038/nrdp.2016.78
Method Description
Homovanillic Acid:
Homovanillic acid (HVA) is measured by solid-phase extraction (SPE) of a 1-mL aliquot of urine. A known amount of stable isotope-labeled HVA internal standard (IS) is added to each urine specimen prior to SPE. HVA and IS are eluted from the SPE column with methanol. The methanol is evaporated and the HVA and IS are redissolved in liquid chromatography tandem-mass spectrometry (LC-MS/MS) mobile phase. A portion of this prepared extract is injected onto a LC column that separates HVA and IS from the bulk of any remaining specimen matrix. The HVA and IS are measured by mass spectrometry/tandem-mass spectrometry using the selected reaction monitoring mode. HVA is quantified using the ratio to IS versus urine calibrators.(Magera MJ, Stoor A, Helgeson JK, Matern D, Rinaldo P. Determination of homovanillic acid in urine by stable isotope dilution and electrospray tandem mass spectrometry. Clin Chim Acta. 2001;306[1-2]:35-41; Eisenhofer G, Grebe S, Cheung NV. Monoamine-producing tumors. In: Rifai N, Horvath AR, Wittwer CT, eds. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 6th ed. Elsevier; 2018:chap 63)
Vanillylmandelic Acid:
Vanillylmandelic acid (VMA) is measured by SPE of a 1-mL aliquot of urine. A known amount of stable isotope-labeled VMA IS is added to each urine specimen prior to SPE. VMA and IS are eluted from the SPE column with methanol. The methanol is evaporated and the VMA and IS are redissolved in LC-MS/MS mobile phase. A portion of this prepared extract is injected onto a LC column that separates VMA and IS from the bulk of any remaining specimen matrix. The VMA and IS are measured by MS/MS using the selected reaction monitoring mode. VMA is quantified using the ratio to IS versus urine calibrators.(Magera MJ, Thompson AL, Stoor AL, et al. Determination of vanillylmandelic acid in urine by stable isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2003;49:825-826; Eisenhofer G, Grebe S, Cheung NV. Monoamine-producing tumors. In: Rifai N, Horvath AR, Wittwer CT, eds. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 6th ed. Elsevier; 2018:chap 63)
Day(s) Performed
Monday through Friday
Report Available
3 to 5 daysSpecimen Retention Time
7 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
83150-HVA
84585-VMA
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| VH | VMA and HVA, Random, U | 90250-2 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 2143 | Vanillylmandelic Acid, VH | 3124-5 |
| 2144 | Homovanillic Acid, VH | 11146-8 |