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, headache, pallor, palpitations);

-high levels of blood or urinary catecholamines or metanephrines.

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

-patients performing DEXA scan are adults above 25 and below 45 years of age.

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

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

EXCLUSION CRITERIA:

Children of age less than 18 years are excluded from F-FLT PET scanning

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.

Glucagon and clonidine testing are not performed in pregnant women.

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

Patients with impaired mental capacity that precludes informed consent.

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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) National Institutes of Health BG 10-CRC RM 1-3140 MSC 1109 10 CENTER DR BETHESDA MD 20892-1109 (301) 402-4594 karel@mail.nih.gov	Karen T. Adams, C.R.N.P. National Institute of Child Health and Human Development (NICHD) National Institutes of Health 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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