Diagnosis of Pheochromocytoma
|First Received Date ICMJE||March 2, 2000|
|Last Updated Date||November 7, 2015|
|Start Date ICMJE||February 2000|
|Primary Completion Date||Not Provided|
|Current Primary Outcome Measures ICMJE
||To investigate the use of radiopharmaceutical tracer, F(18)-FLT for PET/CT scan in evaluating cellular proliferative behavior of various genetically inherited and sporadic pheochromocytomas and paragagliomas in adult patients.|
|Original Primary Outcome Measures ICMJE||Not Provided|
|Change History||Complete list of historical versions of study NCT00004847 on ClinicalTrials.gov Archive Site|
|Current Secondary Outcome Measures ICMJE||Not Provided|
|Original Secondary Outcome Measures ICMJE||Not Provided|
|Current Other Outcome Measures ICMJE||Not Provided|
|Original Other Outcome Measures ICMJE||Not Provided|
|Brief Title ICMJE||Diagnosis of Pheochromocytoma|
|Official Title ICMJE||Diagnosis, Pathophysiology, and Molecular Biology of Pheochromocytoma and Paraganglioma|
The goal of this study is to develop better methods of diagnosis and treatment for pheochromocytomas. These tumors, which usually arise from the adrenal glands, are often difficult to detect with current methods. Pheochromocytomas release chemicals called catecholamines, causing high blood pressure. Undetected, the tumors can lead to severe medical consequences, including stroke, heart attack and sudden death, in situations that would normally pose little or no risk, such as surgery, general anesthesia or childbirth.
Patients with pheochromocytoma may be eligible for this study. Candidates will be screened with a medical history and physical examination, electrocardiogram, and blood and urine tests. Study participants will undergo blood, urine, and imaging tests, described below, to detect pheochromocytoma. If a tumor is found, the patient will be offered surgery. If surgery is not feasible (for example, if there are multiple tumors that cannot be removed), evaluations will continue in follow-up visits. If the tumor cannot be found, the patient will be offered medical treatment and efforts to detect the tumor will continue. Diagnostic tests may include the following:
PLEASE NOTE: Until further notice, we are not offering MIBG131 at this time.
|Detailed Description||Pheochromocytomas/paragangliomas are rare but clinically important chromaffin cell tumors that typically arise from the adrenal gland and constitute a surgically correctable cause of chronic hypertension. The clinical features and consequences of pheochromocytoma/paraganglioma result from the release of catecholamines (e.g., norepinephrine and epinephrine) by the tumor. If a pheochromocytoma/paraganglioma is undetected, stimuli that normally would not pose a hazard, such as surgery, childbirth, or general anesthesia, can evoke catecholamine secretion by the tumor, with clinically significant and even catastrophic outcomes. The diagnosis of pheochromocytoma/paraganglioma and its localization can be challenging, because measurements of plasma levels or urinary excretion of catecholamines and their metabolites and radio-iodinated metaiodobenzylguanidine (MIBG) scanning can yield false-negative results in patients harboring the tumor. Computed tomographic (CT) and magnetic resonance imaging (MRI) lack sufficient specificity. The molecular mechanisms by which genotypic changes predispose to development of pheochromocytoma/paraganglioma remain unknown, even in patients with identified mutations. Moreover, pheochromocytomas/paragangliomas in patients with hereditary predispositions differ in terms of their growth, malignant potential, catecholamine phenotype, and responses to standard screening tests such as glucagon stimulation and clonidine suppression tests. This protocol focuses on molecular and genetic studies that may elucidate the bases for predisposition to develop pheochromocytomas/paragangliomas and for expression of different neurochemical phenotypes and malignant potentials, new imaging approaches, based on [(18)F]-6F-dopamine ([(18)F]-6F-DA), and [(18)F]-L-3,4-dihydroxyphenylalanine ((18)F]-DOPA) positron emission tomographic (PET) scanning, [99m]Tc-methoxyisobutylisonitrile SPECT scintigraphy (99m Tc-MIBI), and new biochemical diagnostic criteria, based on measurement of plasma metanephrines. We also want to evaluate the benefits romidepsin pretreatment for uptake enhancement of [(123/131)I]-MIBG in pheochromocytoma/paraganglioma tumors.|
|Study Type ICMJE||Observational|
|Study Design ICMJE||Not Provided|
|Target Follow-Up Duration||Not Provided|
|Sampling Method||Not Provided|
|Study Population||Not Provided|
|Intervention ICMJE||Not Provided|
|Study Group/Cohort (s)||Not Provided|
* Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
|Recruitment Status ICMJE||Recruiting|
|Estimated Enrollment ICMJE||2000|
|Completion Date||Not Provided|
|Primary Completion Date||Not Provided|
|Eligibility Criteria ICMJE||
Patients are adults or children with known or suspected sporadic or familial pheochromocytoma/paraganglioma, on the basis of one or more of the following:
Signed informed consent is required.
Patients must be willing to return to NIH for follow-up evaluation.
Patients with pheochromocytoma/paraganglioma will be accepted through clinician or self referrals.
Imaging studies are not done in pregnant or lactating women. A pregnancy test is performed in women of child-bearing age (up to age 55). In those with positive results, no PET scanning, MIBG scanning, contrast CT, DCE-MRI, vena cava sampling, glucagon and clonidine testing is performed.
Women who are pregnant are excluded from admission to the Clinical Center but may be studied as outpatients.
Imaging studies are not done in patients that have the following exclusion criteria:
Additionally DCE-MRI is not done in patients with acute or chronic renal insufficiency since gadolinium chelate injection is contraindicated in those patients. In patients where DCE-MRI is considered, a creatinine clearance measurement is performed as a clinically indicated test by the Department of Laboratory Medicine at the NIH Clinical Center. Patients with impaired kidney function will not undergo DCE-MRI. DCE-MRI is also not done in patients with severe claustrophobia or the presence of iron or metal in the MRI scan site, in patients with pacemakers or defibrillators, and in patients with an allergy to gadolinium.
Exclusion criteria for research PET imaging in children
Children of less than 10 years of age,
Children with impaired mental capacity that precludes informed assent,
Pregnant or lactating female adolescents,
Inability to lie still for the entire imaging time (e.g. cough, turbulent children, severe claustrophobia, etc.).
|Ages||7 Years and older|
|Accepts Healthy Volunteers||Yes|
|Listed Location Countries ICMJE||United States|
|Removed Location Countries||Netherlands|
|NCT Number ICMJE||NCT00004847|
|Other Study ID Numbers ICMJE||000093, 00-CH-0093|
|Has Data Monitoring Committee||Not Provided|
|Responsible Party||National Institutes of Health Clinical Center (CC) ( Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) )|
|Study Sponsor ICMJE||Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)|
|Collaborators ICMJE||Not Provided|
|Information Provided By||National Institutes of Health Clinical Center (CC)|
|Verification Date||February 2015|
ICMJE Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP