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Other Rare Unusual Cancers of Childhood

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    The risk of pheochromocytoma or paraganglioma is increased by having any of the following inherited syndromes or gene changes:

    • Multiple endocrine neoplasia 1 syndrome.
    • Multiple endocrine neoplasia 2 syndrome (MEN2A and MEN2B).
    • von Hippel-Lindau disease (VHL).
    • Neurofibromatosis type 1 (NF1).
    • Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST]).
    • Carney triad (paraganglioma, GIST, and pulmonary chondroma).
    • Changes in certain genes including SDHD, SDHB, SDHA, and TMEM127.

    More than half of the children and adolescents diagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increased the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.

    Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test or procedure is done for another reason. Symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other symptoms may be caused by pheochromocytoma and paraganglioma. Other conditions may cause the same symptoms. Check with your child's doctor if you see any of the following problems in your child:

    These symptoms may come and go but high blood pressure is more likely to occur for long periods of time in young patients. These symptoms may also occur with physical activity, injury, anesthesia, surgery to remove the tumor, eating foods such as chocolate and cheese, or passing urine (if the tumor is in the bladder).

    Tests used to diagnose and stage pheochromocytoma and paraganglioma depend on the symptoms and the patient's family history. They may include:

    • Physical exam and history.
    • PET scan.
    • CT scan (CAT scan).
    • MRI (magnetic resonance imaging).

    See the General Information section for a description of these tests and procedures.

    Other tests and procedures used to diagnose pheochromocytoma and paraganglioma include the following:

    • Plasma-free metanephrines test: A blood test that measures the amount of metanephrines in the blood. Metanephrines are substances that are made when the body breaks down adrenaline or noradrenaline. Pheochromocytomas and paragangliomas can make large amounts of adrenaline and noradrenaline and cause high levels of metanephrines in both the blood and urine.
    • Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines (adrenaline or noradrenaline) released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma or paraganglioma.
    • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines (adrenaline or noradrenaline) or metanephrines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts may be a sign of pheochromocytoma or paraganglioma.
    • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
    • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
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