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Study of Adrenal Gland Tumors

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00005927
Recruitment Status : Completed
First Posted : June 29, 2000
Last Update Posted : December 3, 2021
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Information provided by (Responsible Party):
National Institutes of Health Clinical Center (CC) ( National Institute on Deafness and Other Communication Disorders (NIDCD) )

Brief Summary:

The adrenal glands, located atop the kidneys, normally produce several types of hormones. Tumors of these glands may or may not secrete hormones. It is not known what causes these tumors or why some secrete hormones and others do not. Some of the tumors are benign and confined to the adrenal gland, and others are malignant (cancerous), and can spread to other parts of the body. This study will investigate how adrenal gland tumors develop, why some secrete steroid hormones and others do not, and why some are benign and others malignant.

Patients between 3 and 70 years old with a known or suspected adrenal gland tumor may be eligible for this study. Participants will be hospitalized for 7 to 10 days for various tests and procedures that may include the following:

  1. Medical history and physical examination, including body measurements, as appropriate. Children and adolescents will have Tanner staging, including examination of the genitals, to determine the extent of sexual maturity.
  2. 24-hour urine collection to measure hormones in the urine.
  3. Imaging studies, including magnetic resonance imaging (MRI) of the brain, computed tomography (CT) and other X-ray studies.
  4. Blood tests to see if the tumor secretes hormones in response to specific stimuli, including exercise, food, and various hormones. The hormones are given through an intravenous catheter, or IV a thin plastic tube inserted into an arm vein. After the stimulus, blood is drawn through the same IV every 30 minutes for up to 3 hours to measure hormone levels. Based on the results of these tests, some patients may have additional blood tests to check hormone response to special foods, an IV salt solution, or other hormones or drugs given either IV or by mouth (in pill form).
  5. Photographs to document the effects on the body of abnormal hormone secretion from the adrenal tumor.
  6. Small samples of blood and tumor tissue for research and DNA (genetic) analysis.

A discussion of treatment options will be based on the results of tests. If surgery to remove the tumor is recommended, the procedure can be done at NIH under this study protocol. If a malignant tumor is found that cannot be treated surgically, chemotherapy or radiation therapy may be recommended. These options are not offered under this protocol, but may be available under a different NIH study (for example, at the National Cancer Institute). Referrals will be made at the patient s request.

Patients who had surgery may be followed at the NIH outpatient clinic for 1 year after surgery. Patients with certain types of tumors may continue to be followed at NIH once a year for up to 5 years.

A registry of study participants will be created to keep records and correlate medical histories with tissues kept at NIH. The registry will also be used to inform participants of research studies they may be interested in. No individuals or organizations outside of NIH will have access to the registry.

Condition or disease
Adrenal Gland Neoplasm

Detailed Description:
The adrenal glands are the major source in the body of the steroid hormones. In normal physiology, the pituitary hormone ACTH regulates the secretion of glucocorticoids, while the secretion of mineralocorticoids such as aldosterone is controlled by the renin-angiotensin system. In addition to these two classes of steroids, the adrenal gland secretes lesser amounts of intermediate metabolites as well as dehydroepiandrosterone (DHEA) and its sulfated product (DHEAS) and androstenedione, testosterone, estrogen, and estrone. Dysregulated secretion of any of these hormones can be caused by sporadic adrenocortical adenomas or carcinomas, with the development of specific clinical syndromes depending on the identity of the hormones secreted. In at least a subset of cortisol-producing adrenocortical neoplasms, the presence of ectopic or abnormal receptors has been described, resulting in the regulation of cortisol and/or aldosterone by non-physiologic stimuli. The present study will serve as a mechanism to investigate individuals with steroid hormone-secreting adrenocortical tumors of all types for the purpose of identifying hereditary, congenital, or acquired defects leading not only to hormone oversecretion, but also to tumor formation. One of the first goals of the study was (until very recently) to examine the prevalence of ectopic receptor expression in cortisol- and/or aldosterone- hormone secreting adrenocortical tumors. This led to the understanding of the ontogeny of these tumors and the development of novel therapeutic strategies (e.g., receptor antagonists) to control hormone oversecretion. We currently use this information for the evaluation and treatment of our patients. An important research goal of the study is to identify novel genetic defects leading to tumors of the adrenal gland. This is done through a set of methods, from sequencing of the collected DNA to analysis of the expression of large sets of genes using gene array/gene chip analysis. This information may help to identify patients who would benefit from more aggressive intervention strategies, especially those with potentially malignant tumors. This study also provides the patient cohort necessary for the establishment of a bank of tissues of varying tumors of the adrenal cortex, which may serve in the future as an experimental resource to test new diagnostic and therapeutic methods. Finally, an important and more recent goal of this study is to investigate the effects of excess aldosterone on renal, cardiac, metabolic, and bone systems in patients with primary hyperaldosteronism, an important subgroup of patients with adrenocortical tumors. Patients with hyperaldosetronism have not been studied with the same rigor as patients with hypercortisolism in the past; this study aims at investigating the relative contribution of hyperaldosteronism in the etiopathogenesis of a number of clinical problems in patients with adrenocortical lesions and hypertension.

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Study Type : Observational
Actual Enrollment : 690 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Clinical and Molecular Analysis of ACTH-Independent Steroid Hormone Production in Adrenocortical Tissue
Actual Study Start Date : July 1, 2002

Resource links provided by the National Library of Medicine

Hyperaldosteronism and cushing participants
Subjects with Hyperaldosteronism and cushing. Adults, pediatric subjects and family members (DNA collection only for family members).

Primary Outcome Measures :
  1. To study the contribution of ectopic/abnormal membrane hormone receptor expression in the adrenal gland to the pathogenesis of primary adrenocortical hormone over-secretion syndromes [ Time Frame: This is an ongoing project ]
    -to define the germline and/or somatic genetic mutations causing ACTH-independent ushing s syndrome; -to define the biochemicalfunctionality of mutations in adrenocortical tumor; -to investigate novel therapeutic strategies to control hormone oversecretion of biochemically active adrenal tumors

Secondary Outcome Measures :
  1. To investigate the renal, cardiac, metabolic, and bone effects of hyperaldosteronism. To utilize high-density gene array analysis to identify patterns of gene expression that will discriminate between benign and malignant adrenal tumors. [ Time Frame: This is an ongoing project ]
    -to investigate co-secretion of other steroids; -to investigate cardiac, renal, metabolic and coagulopathic consequences of primary aldosteronism; -to investigate the clinical utility of other biomarkers in classifying adrenal disorders

Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.

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Ages Eligible for Study:   3 Years to 70 Years   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Research subjects who present with Adrenal adenoma, adrenal carcinoma, Cushing syndrome, virilizing adrenals tumor, feminizing adrenal tumor, massive macronodular adrenocortical disease, ACTH-independent macronodular adrenal hyperplasia

Patients are adults or children with evidence for the existence of a tumor of the adrenal glands, as indicated by previously obtained imaging studies and/or biochemical investigation of hormonal secretion. This condition is meant to include the possibility of individuals with "sub-clinical" hormone secretion syndromes, which may be detectable at the biochemical level even in the absence of frank clinical signs/symptoms.

All eligible patients are invited to participate in this protocol, regardless of sex, race or ethnic origin. All populations appear at risk for adrenal tumors, and therefore the subject population can include Native Americans, Asian/Pacific Islanders, Caucasian, Hispanic, and Black individuals. Patients will be accepted for evaluation based on referral from clinicians, or may be self-referred, if they can provide evidence supportive of the diagnosis of hormone over-secretion.

Patients must be willing to return to the NIH for follow-up evaluation.

Patients may withdraw from the study at any time.


  1. Children less than 3 years old will be excluded from the protocol because of the limited resources available at the NIH for the care of infants of this age.
  2. Individuals over the age of 70 years of age will be excluded because of the possibility of comorbidities that may significantly affect appropriate initial work-up and post-operative management. In addition, research data may be compromised by the inability to interpret data collected from patients over the age of 70 years that may be on multiple medications for a variety for reasons.
  3. Women who are pregnant or nursing will be excluded from the hyperaldosteronism arm of the protocol. Women with adrenal tumors secreting cortisol or other adrenal hormones may benefit clinically from evaluation and treatment of their tumor, and will be considered for enrollment when clinically indicated.
  4. Individuals whose medical status will not allow them, for safety reasons, to participate in the provocative testing or who have unacceptably high risk for surgical morbidity and mortality will be excluded from the protocol, as they will not be able to participate profitably in the research aspects of this protocol.
  5. Individuals found to have an known inherited syndrome as the cause for hormone oversecretion will be excluded from participation in this protocol, as the mechanisms of hormone oversecretion and tumorigenesis is likely to be distinct in these individuals. Specific examples of syndromes to may be excluded from this protocol include individuals with Carney Complex, McCune-Albright syndrome, and MEN-1. If inquiries are received from such patients, they will be referred to the appropriate ongoing protocols, if possible.

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00005927

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United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
National Institute on Deafness and Other Communication Disorders (NIDCD)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
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Principal Investigator: Crystal Dawn C Kamilaris, M.D. Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Additional Information:
Publications automatically indexed to this study by Identifier (NCT Number):

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Responsible Party: National Institute on Deafness and Other Communication Disorders (NIDCD) Identifier: NCT00005927    
Other Study ID Numbers: 000160
First Posted: June 29, 2000    Key Record Dates
Last Update Posted: December 3, 2021
Last Verified: July 1, 2021
Keywords provided by National Institutes of Health Clinical Center (CC) ( National Institute on Deafness and Other Communication Disorders (NIDCD) ):
Macronodular Adrenals
Cushing's Syndrome
Adrenal Gland
Additional relevant MeSH terms:
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Adrenal Gland Neoplasms
Endocrine Gland Neoplasms
Neoplasms by Site
Adrenal Gland Diseases
Endocrine System Diseases