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Protocol Details

Diagnosis, Pathophysiology, and Molecular Biology of Pheochromocytoma and Paraganglioma

This study is currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number

00-CH-0093

Sponsoring Institute

National Institute of Child Health and Human Development (NICHD)

Recruitment Detail

Type: Participants currently recruited/enrolled
Gender: Male & Female
Min Age: 7
Max Age: N/A

Referral Letter Required

No

Population Exclusion(s)

None

Special Instructions

Currently Not Provided

Keywords

Neurofibromatosis;
Multiple Endocrine Neoplasia (MEN);
von Hippel-Lindau Disease;
Norepinephrine;
Epinephrine;
Metanephrines;
PET;
Paraganglioma

Recruitment Keyword(s)

Pheochromocytoma

Condition(s)

Pheochromocytoma

Investigational Drug(s)

18F - Fluorodopamine
18F - Fluorodopa

Investigational Device(s)

None

Intervention(s)

None

Supporting Site

National Institute of Child Health and Human Development

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:

1. Blood tests - Two blood tests glucagon stimulation and clonidine suppression are done that require insertion of intravenous (i.v.) catheters (thin flexible tubes) into arm veins. While the patient rests lying down, a drug (glucagon or clonidine) is given through the i.v. line. Blood pressure and heart rate are monitored frequently, and blood is collected from the i.v. line to measure levels of catecholamines and their breakdown products, metanephrines.

2. Regional venous sampling - Selective vena caval sampling may be required for some patients. A catheter is placed into a large blood vessel called the inferior vena cava, through which blood circulating in the body returns to the heart. Blood samples are collected for measurement of catecholamines and metanephrines.

3. Standard imaging tests - Non-investigational imaging tests include computed tomography (CT), magnetic resonance imaging (MRI), sonography, and 131I-MIBG scanning. These scans may be done before and after surgical removal of pheochromocytoma.

4. PET imaging - Positron emission tomography (PET) scanning is done using an injection of a radioactive catecholamine called fluorodopamine. The fluorodopamine enters pheochromocytoma cells, making the tumor radioactive and visible on the PET scan. The scan takes up to about 2 hours.

5. Urine - A 24-hour urine collection is collected for analysis.

6. Genetic testing A small blood sample is collected for DNA analysis.

PLEASE NOTE: Until further notice, we are not offering MIBG131 at this time.

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Eligibility

INCLUSION CRITERIA:

Patients are adults or children with known or suspected sporadic or familial pheochromocytoma/paraganglioma, on the basis of one or more of the following:

-New onset of hypertension or hypertensive episodes and symptoms suggestive of pheochromocytoma/paraganglioma (sweating, headaches, pallor, palpitations).

-High levels of blood or urinary catecholamines, metanephrines, or methoxytyramine.

-Personal or family history of pheochromocytoma/paraganglioma or genetic mutations known to predispose individuals to develop pheochromocytoma/paraganglioma.

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.

EXCLUSION CRITERIA:

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:

-Pregnant or lactating women,

-Patients with impaired mental capacity that precludes informed consent,

-Patients with a body weight of greater than or equal to 400 pounds due to weight limitations of the scanner or patients who are not able to enter the bore of the PET/CT scanner due to BMI,

-Inability to lie still for the entire imaging time (e.g. cough, severe arthritis, etc.),

-Inability to complete the needed investigational and standard-of-care imaging examinations due to other reasons (severe claustrophobia, radiation phobia, etc.),

-Any additional medical condition, serious illness, or other extenuating circumstance that, in the opinion of the Investigator, may significantly interfere with study compliance.

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.).


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Citations:

Histopathology of benign versus malignant sympathoadrenal paragangliomas: clinicopathologic study of 120 cases including unusual histologic features

Multiple endocrine neoplasia type 2: clinical features and screening

The clinical and screening age-at-onset distribution for the MEN 2 syndrome

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Contacts:

Principal Investigator

Referral Contact

For more information:

Karel Pacak, M.D.
National Institute of Child Health and Human Development (NICHD)
BG 10-CRC RM 1-3140
10 CENTER DR
BETHESDA MD 20814
(301) 402-4594
karel@mail.nih.gov

Karen T. Adams, C.R.N.P.
National Institute of Child Health and Human Development (NICHD)
BG 10-CRC RM 1-3140
10 CENTER DR
BETHESDA MD 20814
(301) 402-7785
adamskt@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:

NCT00004847

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