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Mayo Test ID VMAR Vanillylmandelic 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 this purpose are:

-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 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 random urine pH to a level between 1 and 5 by adding 50% acetic acid or hydrochloric acid dropwise and checking the pH.


Useful For

Screening children for catecholamine-secreting tumors using a random urine collection when requesting vanillylmandelic acid only

 

Supporting a diagnosis of neuroblastoma

 

Monitoring patients with a treated neuroblastoma

Method Name

Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)

Reporting Name

Vanillylmandelic Acid, Random, U

Specimen Type

Urine

Specimen 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

Vanillylmandelic acid (VMA) and other catecholamine metabolites (homovanillic acid [HVA] and dopamine) are typically elevated in patients with catecholamine-secreting tumors (eg, neuroblastoma, pheochromocytoma, and other neural crest tumors). VMA and HVA levels may also be useful in monitoring patients who have been treated as a result of one of the above-mentioned tumors.

Reference Values

<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 (adults): <7.0 mg/g creatinine

Interpretation

Vanillylmandelic acid (VMA) and/or homovanillic acid 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 VMA values are suggestive of a pheochromocytoma, but they are not diagnostic.

Cautions

Values are more commonly elevated during a hypertensive episode.

 

Values may be normal in some individuals with a pheochromocytoma.

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. Hyland K. Disorders of neurotransmitter metabolism. In: Blau N, Duran M, Blaskovics ME, Gibson KM, eds. Physician's Guide to the Laboratory Diagnosis of Metabolic Diseases. Springer; 2003:107-122

3. 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

4. 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

5. 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

6. Matthay KK, Maris JM, Schleiermacher G, et al. Neuroblastoma. Nat Rev Dis Primers. 2016;2:16078. doi:10.1038/nrdp.2016.78

Method Description

Vanillylmandelic acid (VMA) is measured by solid-phase extraction (SPE) of a 1-mL aliquot of urine. A known amount of stable-isotope labeled VMA internal standard (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 liquid chromatography tandem mass spectrometry 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 tandem-mass spectrometry 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 days

Specimen Retention Time

7 days

Performing 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

84585

LOINC Code Information

Test ID Test Order Name Order LOINC Value
VMAR Vanillylmandelic Acid, Random, U 3124-5

 

Result ID Test Result Name Result LOINC Value
60274 Vanillylmandelic Acid, Random, U 3124-5