Protocol Number: 00-CH-0093

Title:

Diagnosis, Pathophysiology, and Molecular Biology of Pheochromocytoma

Number:

00-CH-0093

Summary:

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.

Sponsoring Institute:

National Institute of Child Health and Human Development (NICHD)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

Patients are adults or children with known or suspected sporadic or familial pheochromocytoma, on the basis of one or more of the following: (a) new onset of hypertension or hypertensive episodes and symptoms suggestive of pheochromocytoma (sweating, headache, pallor, palpitations); or (b) high levels of blood or urinary catecholamines or metanephrines.

Both male and female subjects of all ethnic and racial groups are eligible.

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

Patients with pheochromocytoma will be accepted based on referral from clinicians.

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, or vena cava sampling is performed.

Pregnant women who are greater than 26 weeks pregnant are excluded from admission to the Clinical Center, but may be studied as outpatients.

[18F]-6F-DA PET scanning is not done in children.

Patients with impaired mental capacity that precludes informed consent.

Special Instructions: Currently Not Provided

Keywords:

Neurofibromatosis

Multiple Endocrine Neoplasia (MEN)

von Hippel-Lindau Disease

Norepinephrine

Epinephrine

Metanephrines

PET

Paraganglioma

Recruitment Keywords:

Pheochromocytoma

Conditions:

Pheochromocytoma

Investigational Drug(s):

Fluorodopamine

Investigational Device(s):

None

Contacts:

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

Electronic Mail:prpl@mail.cc.nih.gov

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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