Screening children for catecholamine-secreting tumors using a 24-hour urine collection when requesting homovanillic acid only
Monitoring neuroblastoma treatment
Screening patients with possible inborn errors of catecholamine metabolism
Homovanillic acid (HVA) measurement in urine is used for screening children for catecholamine-secreting tumors, such as neuroblastoma, pheochromocytoma, and other neural crest tumors, and monitoring those who have had treatment for these tumors.
HVA measurement is also useful for diagnosing children with disorders of catecholamine metabolism, such as monoamine oxidase-A deficiency and dopamine beta-hydroxylase deficiency, which result in either decreased or elevated urinary HVA values, respectively.
Treatment with L-dopa can impact test results and should be discontinued 24 hours prior to collection. Bactrim can impact test results and should be noted at time of collection.
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
HVA (Homovanillic Acid)
Neuroblastoma Profile
Urine
1. Collection duration and urine volume in milliliters are required.
2. Patient's age is required.
3. All patients receiving L-dopa should be identified to the laboratory when this test is ordered.
4. Bactrim may interfere with detection of the analyte. All patients taking Bactrim should be identified to the laboratory when this test is ordered.
Question ID | Description | Answers |
---|---|---|
TM39 | Collection Duration | |
VL37 | Urine Volume |
Patient Preparation: Administration of L-dopa may falsely increase homovanillic acid results; it should be discontinued 24 hours prior to and during specimen collection.
Supplies: Urine Tubes, 10 mL (T068)
Container/Tube: Plastic, 10-mL urine tube
Specimen Volume: 5 mL
Collection Instructions:
1. Add 25 mL of 50% acetic acid as preservative at start of collection. If specimen is refrigerated during collection, preservative may be added up to 12 hours after collection. Use 15 mL of 50% acetic acid for children younger than 5 years. This preservative is intended to achieve a pH of between approximately 1 and 5.
2. Collect a 24-hour urine specimen.
3. If necessary, adjust urine pH to a level between 1 and 5 by adding 50% acetic acid or hydrochloric acid dropwise and checking the pH.
Additional Information:
1. The sensitivity of this test is greater on a 24-hour specimen than on a random specimen.
2. See Urine Preservatives-Collection and Transportation for 24-Hour Urine Specimens for multiple collections.
If not ordering electronically, complete, print, and send an Oncology Test Request (T729) with the specimen.
Note: For preservative or application of temperature controls timing, see Specimen Required.
Ambient (No additive) | No |
Refrigerate (No additive) | No |
Frozen (No additive) | No |
50% Acetic Acid | Preferred |
Boric Acid | No |
Diazolidinyl Urea | No |
6M Hydrochloric Acid | OK |
6M Nitric Acid | OK |
Sodium Carbonate | No |
Thymol | No |
Toluene | No |
2 mL
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Urine | Refrigerated (preferred) | 28 days | |
Frozen | 180 days |
Screening children for catecholamine-secreting tumors using a 24-hour urine collection when requesting homovanillic acid only
Monitoring neuroblastoma treatment
Screening patients with possible inborn errors of catecholamine metabolism
<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
> or =15 years (adults): <8.0 mg/24 hours
Vanillylmandelic acid (VMA) and/or homovanillic acid (HVA) concentrations are elevated in over 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 values are suggestive of a deficiency of dopamine beta-hydrolase, a neuroblastoma, a pheochromocytoma, or may reflect administration of L-dopa.
Decreased urinary HVA values may suggest monoamine oxidase-A deficiency.
Administration of L-dopa may falsely increase homovanillic acid (HVA) results. Patients receiving L-dopa should stop taking it for 24 hours before and during the collection.
All patients receiving L-dopa should be identified to the laboratory when vanillylmandelic acid (VMA) and HVA tests are ordered.
All patients taking Bactrim should be identified to the laboratory when VMA and HVA tests are ordered due to potential interference.
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
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)
Monday through Friday
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.
83150
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
HVA | Homovanillic Acid (HVA), 24 Hr, U | 13760-4 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
3572 | Homovanillic Acid, Adult (>14 yr) | 2436-4 |
3573 | Homovanillic Acid, Child (<15 yr) | 13760-4 |
TM39 | Collection Duration | 13362-9 |
VL37 | Urine Volume | 3167-4 |