Test Id : ACC

Testing begins with a clinical risk assessment based on clinical data before integration with biochemical steroid data to assess the probability of a malignant adrenal cortical carcinoma (ACC) or other malignancy (sarcoma, lymphoma, other) as well as the probability of a benign mass (adenoma, myelolipoma, cyst, other).

Clinical data includes age at diagnosis, gender, mode of discovery and hormonal status along with tumor diameter and an unenhanced CT density measurement of the tumor (in Hounsfield units).

Steroids and their metabolites are extracted, analyzed, quantitated, and reported. Each reported analyte also includes a Z-score. An integrated risk assessment based on clinical data in combination with biochemical steroid data is reported to assess the probability of a malignant ACC or other malignancy as well as the probability of a benign mass.

See Adrenal Mass Panel Clinical Data Definition of Malignancy Predictors in Special Instructions.

Ship specimens frozen.

Collection duration and 24-hour volume is required.

The following information is required (NA or Not Applicable are not acceptable responses):

-Age at diagnosis (not offered for pediatric patients)

-Gender

-Mode of discovery (incidental, cancer staging, other)

-Tumor diameter (mm)

-Unenhanced CT (Hounsfield units)

-Hormonal excess (Yes, No)

If information is not provided, testing may be delayed or canceled.

If not ordering electronically, Adrenal Mass Panel Patient Information is required. See Special Instructions.

Supplies: Aliquot Tube, 5 mL (T465)

Container/Tube: Plastic urine tube

Specimen Volume: 4 mL

Collection Instructions:

1. Collect urine for a full 24 hours (required) and record the total volume.

2. Do not add preservatives. Specimens containing preservatives will be canceled.

3. Entire 24 hour collection must be mixed well prior to aliquoting into a 5 mL plastic tube.

• Urine Preservatives-Collection and Transportation for 24-Hour Urine Specimens
• Adrenal Mass Panel Clinical Data Definition of Malignancy Predictors
• Adrenal Mass Panel Patient Information

Adrenal Mass Panel Patient Information is required, see Special Instructions.

Note: The application of temperature controls must occur within 4 hours of completion of the collection.

2 mL

Aids in assessing malignancy in adrenal masses

May aid in improving diagnostic and prognostic prediction and dissect disease mechanisms for the following applications:

-Diagnostic assessment and follow up of adrenal cortical carcinoma (ACC)

-Differential diagnostic assessment of adrenal tumors

-Additional assessment related to Cushing syndrome, subclinical Cushing syndrome, primary aldosteronism, inborn errors of steroidogenesis, polycystic ovary syndrome

This test is not useful for establishing eligibility for a specific treatment as results must be interpreted in conjunction with the clinical status of the patient.

Testing begins with a clinical risk assessment based on clinical data before integration with biochemical steroid data to assess the probability of a malignant adrenal cortical carcinoma (ACC) or other malignancy (sarcoma, lymphoma, other) as well as the probability of a benign mass (adenoma, myelolipoma, cyst, other).

Clinical data includes age at diagnosis, gender, mode of discovery and hormonal status along with tumor diameter and an unenhanced CT density measurement of the tumor (in Hounsfield units).

Steroids and their metabolites are extracted, analyzed, quantitated, and reported. Each reported analyte also includes a Z-score. An integrated risk assessment based on clinical data in combination with biochemical steroid data is reported to assess the probability of a malignant ACC or other malignancy as well as the probability of a benign mass.

See Adrenal Mass Panel Clinical Data Definition of Malignancy Predictors in Special Instructions.

Approximately 80 million computerized tomography (CT) scans are performed in the United States every year. Adrenal tumors are found incidentally in about 5% of these scans. Most of these tumors will turn out to be benign, but a small fraction will be adrenal cortical carcinoma (ACC), a type of cancer with high mortality and frequent recurrence. Even for localized disease, the 5 year survival rates do not exceed 65%, and distant spread is associated with a greater than 90% mortality rate within that time period. Early diagnosis of a malignant adrenal mass is therefore imperative to assure timely and appropriate therapy.

Unfortunately, CT imaging alone is very limited in its ability to distinguish benign from malignant adrenal tumors since only very small and hypodense lesions can be easily dismissed as benign. The sizeable group of patients with larger or denser tumors ends up with an arduous workup that frequently includes additional imaging studies, hormonal testing, and biopsy. However, even the latter has both a high diagnostic false positive- and false negative rate, and ultimately the tumor is often resected, sometimes unnecessarily. On the other hand, the delays due to the diagnostic work might compromise optimal care for those tumors that prove malignant.

In addition, patients who are believed to probably not have adrenal cancer after their workup, and those who opt out of biopsy or surgery, often still require long-term follow up with regular re-imaging and repeated hormone testing, with resultant radiation exposure and high health care costs.

This adrenal mass panel is a noninvasive and more accurate test to diagnose malignant adrenal tumors, via urinary steroid profiling. It differentiates ACC, a rare and lethal tumor, from benign adrenocortical adenomas (ACA), including those that overproduce corticosteroids, or mineral steroids, or sex steroids, or those that are hormonally inactive. The test utilizes both clinical and laboratory data. The clinical parameters are age at diagnosis and sex of the patient, the size of the tumor by CT scanning and its x-ray density in Hounsfield units, whether it was detected incidentally or not, and whether there is evidence of hormone overproduction. All of this data are readily available for almost all patients with an adrenal mass, and are used by an algorithm to calculate the pretest probability of having ACC. The steroid profile testing is then performed and its results are added into the risk calculation algorithm to generate a posttest probability. The final result will provide the referring physicians a highly accurate probability for ACC and will thereby facilitate the optimal choice of further investigation, if any, based on an informed discussion between doctor and patient.

Understanding the Adrenal Glands

The human body has two adrenal glands, one above each kidney. Adrenal glands influence many processes and functions of our body, mainly through production of three types of steroid hormones:

-Mineralocorticoids (eg, aldosterone, which helps control blood pressure)

-Glucocorticoids (eg, cortisol, which is important for metabolism, immune response and stress)

-Sex Steroids (eg, DHEAS, a precursor of testosterone and estradiol)

These steroids are all synthesized from cholesterol via enzymes in the adrenal glands. In benign ACA, near-normal levels of precursor and bioactive steroids are produced. By contrast, ACC frequently shows abnormal patterns of steroid production. By measuring 25 different steroid metabolites, even subtle abnormalities can be detected. This is the basis for the assessment capability of profiling 25 steroids.

Epidemiology of Adrenal Tumors

Adrenal masses are found in 5% of the population. The prevalence increases with age, to around 10% in 70-year-old patients.

Although the majority of these tumors are benign, around 30% of adrenal tumors (>4cm) are malignant (most represented by ACCs), and the survival rate for these patients is very poor unless detected early.

Note: Due to the wide range of urine steroid metabolite concentrations seen in healthy individuals and their skewed distribution, the reference values are based on the back calculated +/- 3SD of log transformed data.

Males 18-49 years:

Androsterone: 182-29,212 mcg/24 hour

Etiocholanolone: 133-23,272 mcg/24 hour

Dehydroepiandrosterone: <5-81,554 mcg/24 hour

16a-OH-Dehydroepiandrosterone: 13-29,945 mcg/24 hour

5-Pregnenetriol: 23-7,328 mcg/24 hour

5-Pregnenediol: 13-2,823 mcg/24 hour

Tetrahydro-11-Corticosterone: 8-1,961 mcg/24 hour

Tetrahydro-11-Deoxycorticosterone: <5-316 mcg/24 hour

Pregnanediol: 12-3,812 mcg/24 hour

17a-OH-Pregnanolone: 15-2,466 mcg/24 hour

Pregnanetriol: 66-9,409 mcg/24 hour

Pregnanetriolone: <5-550 mcg/24 hour

Tetrahydrodeoxycortisol: 7-1520 mcg/24 hour

Cortisol: <5-903 mcg/24 hour

6B-OH-Cortisol: 13-2,303 mcg/24 hour

Tetrahydrocortisol: 152-22,723 mcg/24 hour

5a-Tetrahydrocortisol: 157-24,059 mcg/24 hour

B-Cortol: 30-5,115 mcg/24 hour

11B-OH-Androsterone: 108-11,987 mcg/24 hour

11B-OH-Etiocholanolone: 22-8,312 mcg/24 hour

Cortisone: 12-842 mcg/24 hour

Tetrahydrocortisone: 271-44,355 mcg/24 hour

a-Cortolone: 140-14,885 mcg/24 hour

B-Cortolone: 72-9,740 mcg/24 hour

11-Oxoetiocholanolone: 70-8,446 mcg/24 hour

Males > or =50 years:

Androsterone: 118-25,389 mcg/24 hour

Etiocholanolone: 127-15,640 mcg/24 hour

Dehydroepiandrosterone: 7-4,260 mcg/24 hour

16a-OH-Dehydroepiandrosterone: 11-6,183 mcg/24 hour

5-Pregnenetriol: 24-2,162 mcg/24 hour

5-Pregnenediol: 17-1,296 mcg/24 hour

Tetrahydro-11-Corticosterone: 16-1,674 mcg/24 hour

Tetrahydro-11-Deoxycorticosterone: <5-297 mcg/24 hour

Pregnanediol: 23-1,846 mcg/24 hour

17a-OH-Pregnanolone: 18-1,747 mcg/24 hour

Pregnanetriol: 115-5,432 mcg/24 hour

Pregnanetriolone: 5-221 mcg/24 hour

Tetrahydrodeoxycortisol: 12-1,277 mcg/24 hour

Cortisol: 12-597 mcg/24 hour

6B-OH-Cortisol: 22-2,406 mcg/24 hour

Tetrahydrocortisol: 331-19,009 mcg/24 hour

5a-Tetrahydrocortisol: 155-35,266 mcg/24 hour

B-Cortol: 56-3,541 mcg/24 hour

11B-OH-Androsterone: 142-13,135 mcg/24 hour

11B-OH-Etiocholanolone: 69-6,805 mcg/24 hour

Cortisone: 24-732 mcg/24 hour

Tetrahydrocortisone: 454-34,576 mcg/24 hour

a-Cortolone: 211-17,591 mcg/24 hour

B-Cortolone: 114-8,434 mcg/24 hour

11-Oxoetiocholanolone: 155-7,174 mcg/24 hour

Females 18-49 years:

Androsterone: 90-29,625 mcg/24 hour

Etiocholanolone: 127-24,568 mcg/24 hour

Dehydroepiandrosterone: <5-12,317 mcg/24 hour

16a-OH-Dehydroepiandrosterone: 5-31,248 mcg/24 hour

5-Pregnenetriol: 17-4,166 mcg/24 hour

5-Pregnenediol: 6-2,900 mcg/24 hour

Tetrahydro-11-Corticosterone: 13-1,548 mcg/24 hour

Tetrahydro-11-Deoxycorticosterone: <5-833 mcg/24 hour

Pregnanediol: 8-44,760 mcg/24 hour

17a-OH-Pregnanolone: 7-3,208 mcg/24 hour

Pregnanetriol: 50-9,768 mcg/24 hour

Pregnanetriolone: <5-139 mcg/24 hour

Tetrahydrodeoxycortisol: 7-1,047 mcg/24 hour

Cortisol: 11-642 mcg/24 hour

6B-OH-Cortisol: 22-2,061 mcg/24 hour

Tetrahydrocortisol: 185-16,515 mcg/24 hour

5a-Tetrahydrocortisol: 45-22,591 mcg/24 hour

B-Cortol: 28-4260 mcg/24 hour

11B-OH-Androsterone: 59-12,462 mcg/24 hour

11B-OH-Etiocholanolone: 32-6,354 mcg/24 hour

Cortisone: 19-749 mcg/24 hour

Tetrahydrocortisone: 262-32,461 mcg/24 hour

a-Cortolone: 207-13,931 mcg/24 hour

B-Cortolone: 63-7,489 mcg/24 hour

11-Oxoetiocholanolone: 63-7,449 mcg/24 hour

Females > or =50 years:

Androsterone: 32-10,134 mcg/24 hour

Etiocholanolone: 52-10,946 mcg/24 hour

Dehydroepiandrosterone: <5-10,046 mcg/24 hour

16a-OH-Dehydroepiandrosterone: <5-9,982 mcg/24 hour

5-Pregnenetriol: 10-1,901 mcg/24 hour

5-Pregnenediol: <5-2,732 mcg/24 hour

Tetrahydro-11-Corticosterone: 14-1,229 mcg/24 hour

Tetrahydro-11-Deoxycorticosterone: <5-123 mcg/24 hour

Pregnanediol: 8-2,138 mcg/24 hour

17a-OH-Pregnanolone: <5-571 mcg/24 hour

Pregnanetriol: 26-3,444 mcg/24 hour

Pregnanetriolone: <5-348 mcg/24 hour

Tetrahydrodeoxycortisol: 8-801 mcg/24 hour

Cortisol: 9-336 mcg/24 hour

6B-OH-Cortisol: 25-1,365 mcg/24 hour

Tetrahydrocortisol: 237-14,050 mcg/24 hour

5a-Tetrahydrocortisol: 92-12,604 mcg/24 hour

B-Cortol: 29-3289 mcg/24 hour

11B-OH-Androsterone: 86-9,280 mcg/24 hour

11B-OH-Etiocholanolone: 40-7,002 mcg/24 hour

Cortisone: 15-555 mcg/24 hour

Tetrahydrocortisone: 359-24,320 mcg/24 hour

a-Cortolone: 125-17,472 mcg/24 hour

B-Cortolone: 82-5,784 mcg/24 hour

11-Oxoetiocholanolone: 78-6,571 mcg/24 hour

Reference values have not been established for patients who are younger than 18 years of age.

Test provides clinical risk values based on clinical data alone as well as integrated risk values based on clinical data in combination with biochemical steroid data. Reported risk values correspond to the probability of a malignant adrenal cortical carcinoma (ACC) or other malignancy (eg, sarcoma, lymphoma) as well as the probability of a benign mass (eg, adenoma, myelolipoma, cyst).

Test results provide the referring physician with probabilities for a variety of outcomes, thereby aiding the interpretation of clinical status and optimal paths for further investigation, if any, based on an informed discussion between provider and patient. Test results should always be interpreted in conjunction with all other clinical findings as they cannot be interpreted as absolute evidence for the presence or absence of malignant disease.

See Adrenal Mass Panel Clinical Data Definition of Malignancy Predictors in Special Instructions.

Test not offered for pediatric patients. Risk assessments based off of adult populations.

Test results cannot be interpreted as absolute evidence for the presence or absence of malignant disease. This test should not form the sole basis for a diagnosis or treatment decision as results must be interpreted within the clinical context of the patient and should always be used in conjunction with clinical findings.

1. Arlt W, Biehl M, Taylor AE, et al: Urine steroid metabolomics as a biomarker tool for detecting malignancy in adrenal tumors. J Clin Endocrinol Metab.2011 Dec;96(12):3775-3784. doi: 10.1210/jc.2011-1565

2. Hines JM, Bancos I, Bancos C, et al: High-Resolution, Accurate-Mass (HRAM) Mass Spectrometry Urine Steroid Profiling in the Diagnosis of Adrenal Disorders. Clin Chem. 2017 Dec;63(12):1824-1835. doi: 10.1373/clinchem.2017.271106

3. Bancos I, Arlt W: Diagnosis of a malignant adrenal mass: the role of urinary steroid metabolite profiling. Curr Opin Endocrinol Diabetes Obes. 2017 Jun;24(3):200-207. doi: 10.1097/MED.0000000000000333

4. Fassnacht M, Arlt W, Bancos I, et al: Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2016 Aug;175(2):G1-G34. doi: 10.1530/EJE-16-0467

• Urine Preservatives-Collection and Transportation for 24-Hour Urine Specimens
• Adrenal Mass Panel Clinical Data Definition of Malignancy Predictors
• Adrenal Mass Panel Patient Information

Steroids and their metabolites are extracted and analyzed with internal standard for detection by liquid chromatography-tandem mass spectrometry, high-resolution accurate mass (LC-MS/MS HRAM).(Unpublished Mayo method)

Clinical predictors and steroid data are algorithmically integrated to give a likelihood of adrenal cortical carcinoma, other malignancy, or benign mass.

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This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

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