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Protocol Details

Clinical and Molecular Analysis of ACTH-Independent Steroid Hormone Production in Adrenocortical Tissue

This study is NOT currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number

00-CH-0160

Sponsoring Institute

National Institute on Deafness and Other Communication Disorders (NIDCD)

Recruitment Detail

Type: Completed Study; data analyses ongoing
Gender: Male & Female
Min Age: 3
Max Age: 70

Referral Letter Required

No

Population Exclusion(s)

None

Special Instructions

Currently Not Provided

Keywords

Macronodular Adrenals;
Cushing's Syndrome;
Adrenal Gland

Recruitment Keyword(s)

None

Condition(s)

Adrenal Gland Neoplasm

Investigational Drug(s)

None

Investigational Device(s)

None

Intervention(s)

None

Supporting Site

National Institute of Child Health and Human DevelopmentNational Institute on Deafness and Other Communication Disorders

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.

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Eligibility

INCLUSION CRITERIA:

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.

EXCLUSION CRITERIA:

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.


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Citations:

Torpy DJ, Stratakis CA, Chrousos GP. Hyper- and hypoaldosteronism. Vitam Horm. 1999;57:177-216.

Dluhy RG, Lifton RP. Glucocorticoid-remediable aldosteronism. J Clin Endocrinol Metab. 1999 Dec;84(12):4341-4.

Torpy DJ, Gordon RD, Lin JP, Huggard PR, Taymans SE, Stowasser M, Chrousos GP, Stratakis CA. Familial hyperaldosteronism type II: description of a large kindred and exclusion of the aldosterone synthase (CYP11B2) gene. J Clin Endocrinol Metab. 1998 Sep;83(9):3214-8.

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Contacts:

Principal Investigator

Referral Contact

For more information:

Crystal Dawn C. Kamilaris, M.D.
National Institute of Child Health and Human Development (NICHD)
BG 10-CRC RM 1-3121
10 CENTER DR
BETHESDA MD 20814
(301) 402-3699
crystal.kamilaris@nih.gov

Crystal Dawn C. Kamilaris, M.D.
National Institute of Child Health and Human Development (NICHD)
BG 10-CRC RM 1-3121
10 CENTER DR
BETHESDA MD 20814
(301) 402-3699
crystal.kamilaris@nih.gov

Office of Patient Recruitment
National Institutes of Health Clinical Center (CC)
Building 61, 10 Cloister Court
Bethesda, Maryland 20892
Toll Free: 1-800-411-1222
Local Phone: 301-451-4383
TTY: 1-866-411-1010
PRPL@cc.nih.gov

Clinical Trials Number:

NCT00005927

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