Protocol Details

New Imaging Modalities in the Evaluation of Patients with Ectopic Cushing's Syndrome

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number	99-CH-0055
Sponsoring Institute	National Institute of Child Health and Human Development (NICHD)
Recruitment Detail	Type: Participants currently recruited/enrolled Gender: Male & Female Min Age: 18 Max Age: 90
Referral Letter Required	No
Population Exclusion(s)	Children
Special Instructions	Currently Not Provided
Keywords	PET; (18F)-DOPA; Pentetreotide; ACTH; Octreotide
Recruitment Keyword(s)	Ectopic Cushing Syndrome
Condition(s)	Cushing Syndrome
Investigational Drug(s)	(18F)-L-3,4-dihydroxyophenylalanine (18F-DOPA)
Investigational Device(s)	None
Intervention(s)	None
Supporting Site	National Institute of Child Health and Human Development

Cushing Syndrome is an endocrine disorder causing an over production of the hormone cortisol. Cortisol is produced in the adrenal gland as a response to the production of ACTH in the pituitary gland.

Between 10% and 20% of patients with hypercortisolism (Cushing Syndrome) have ectopic production of the hormone ACTH. Meaning, the hormone is not being released from the normal site, the pituitary gland. In many cases the ectopic ACTH is being produced by a tumor of the lung, thymus, or pancreas. However, in approximately 50% of these patients the source of the ACTH cannot be found even with the use of extensive imaging studies such as CT scans, MRIs, and nuclear scans (111-indium pentetreotide). The ability of these tests to locate the source of the hormone production is dependent on the changes of anatomy and / or the dose and adequate uptake of the radioactive agent. The inability to detect the source of ectopic ACTH production often results in unnecessary pituitary surgery or irradiation.

Unlike the previously described tests, positron emission tomography (PET scan) has the ability to detect pathologic tissue based on physiologic and biochemical processes within the abnormal tissue.

This study will test whether [18-F]-fluorodeoxyglucose (FDG) or use of a higher dose of [111In-DTPA-D-Phe]-pentetreotide can be used to successfully localize the source of ectopic ACTH production.

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Eligibility

INCLUSION CRITERIA:

All eligible patients are invited to participate in this protocol. Patients are adults with possible ectopic Cushing syndrome. Since both men and women are affected with ectopic Cushing syndrome, both sexes are studied. All ethnic and racial groups are at risk and will be included. Patients must be willing to return to NIH for follow-up studies.

EXCLUSION CRITERIA:

Pregnant or lactating women.

Children (age less than18) are excluded.

Patients with any severe active infection.

Patients with clinically significantly impaired cardiovascular (e.g., history of abnormally low ejection fraction, the presence of moderate pulmonary fluid overload or leg edema, and blood pressure over 190/100), abnormal coagulation (PT and PTT elevated by 30 percent above the normal values), hematopoietic (hematocrit less than 30 percent, hemoglobin below 10 g/dl, white count below 3000 K/UL, and platelets below 100,000 K/mm(3)), hepatic (liver enzymes elevated by 3-fold above normal values) or renal function (plasma creatinine level over 2.0).

Patients with impaired mental capacity or markedly abnormal psychiatric evaluation that precludes informed consent.

Patients with body weight over 136 kg, which is the limit for the tables used in the scanning areas.

Patients with combined blood withdrawal, during the six weeks preceding the study, of greater than 450 ml.

Patients with known allergy to [111In-DTPA-D-Phe]-pentetreotide or other somatostatin analogues.

Patients more than 70 years of age due to other possible existing diseases which may significantly affect appropriate initial work-up and post-operative morbidity and mortality.

Patients with strong evidence for Cushing disease. This includes those with positive IPSS or a lesion on pituitary MRI.

Patients taking medications that alter CYP3A4 activity will not be eligible for the mifepristone study, since this P450 system metabolizes mifepristone.

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Citations:

Occult ectopic secretion of corticotropin

Ectopic ACTH syndrome Diagnostic and therapeutic aspects

The diagnosis and differential diagnosis of Cushing's syndrome

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Contacts:

Principal Investigator	Referral Contact	For more information:
Lynnette K. Nieman, M.D. National Institute of Child Health and Human Development (NICHD) National Institutes of Health BG 10-CRC RM 1-3140 MSC 1109 10 CENTER DR BETHESDA MD 20892-1109 (301) 496-8935 niemanl@mail.nih.gov	Mai Lloyd National Institute of Child Health and Human Development (NICHD) National Institutes of Health Building 10 Room 1-3121 10 Center Drive Bethesda, Maryland 20892 (301) 402-2139 lloydm4@mail.nih.gov	Patient Recruitment and Public Liaison Office Building 61 10 Cloister Court Bethesda, Maryland 20892-4754 Toll Free: 1-800-411-1222 TTY: 301-594-9774 (local),1-866-411-1010 (toll free) Fax: 301-480-9793 prpl@mail.cc.nih.gov

Clinical Trials Number:

NCT00001849

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