GLOSSARY OF TERMS
pheochromocytoma & paraganglioma
Adrenal gland:
A small endocrine gland located above each kidney that produces steroid hormones, adrenaline, and noradrenaline. These hormones play crucial roles in regulating blood pressure, heart rate, and other essential body functions. The adrenal gland is also referred to as the suprarenal gland.
Adrenaline:
A hormone and neurotransmitter, also known as epinephrine, which helps the body respond to stress by increasing heart rate, blood flow, and energy availability.
Alpha blockers:
Medications used to treat high blood pressure.
They work by relaxing muscles and keeping blood vessels open, improving blood flow and reducing blood pressure. These drugs inhibit the hormone norepinephrine’s effect on muscle tightness in blood vessels. They are also referred to as alpha-adrenergic antagonists.
Anesthesiologist:
A medical professional with specialized training in administering drugs or other methods to alleviate pain and ensure patient comfort during surgical or medical procedures.
Beta blockers:
Drugs that help lower blood pressure by blocking the effects of adrenaline (epinephrine). These medications slow the heart rate and decrease the force of the heartbeat, effectively reducing blood pressure. They are also known as beta-adrenergic blocking agents.
Blood catecholamine studies:
A diagnostic procedure that measures the amount of catecholamines (like adrenaline and noradrenaline) in the blood. High levels of these hormones may suggest conditions such as pheochromocytoma or paraganglioma.
Catecholamine:
A type of chemical produced by nerve cells that serves as both a hormone and neurotransmitter, playing a role in the body’s stress response. Examples include dopamine, adrenaline (epinephrine), and noradrenaline (norepinephrine). High levels can cause symptoms like high blood pressure, headaches, and anxiety.
CT Scan:
A diagnostic imaging technique that combines x-rays and computer technology to produce detailed cross-sectional images of internal organs and tissues. It may involve the use of a contrast dye to enhance visibility. This scan is commonly used to diagnose diseases, plan treatments, or evaluate the effectiveness of interventions.
Endocrinologist:
A physician specializing in the diagnosis and treatment of conditions related to the endocrine system, such as hormone imbalances, thyroid disorders, diabetes, and adrenal gland issues.
Gallium Dotatate Scan:
A nuclear medicine imaging procedure used to identify rapidly dividing cells, such as cancer cells. A small amount of radioactive gallium is injected into a vein, and the areas where it accumulates are detected using a specialized scanner.
Hypertension:
A medical condition defined by a blood pressure reading of 140/90 mmHg or higher. It typically has no obvious symptoms but can lead to serious health problems like heart attack, stroke, or kidney failure.
MIBG scan:
A diagnostic test used to locate neuroendocrine tumors, including pheochromocytomas and paragangliomas. A small amount of radioactive MIBG is injected into the bloodstream, where it binds to tumor cells. A scanner detects the radioactive substance to pinpoint the tumor’s location.
MIBG treatment (I31I-MIBG):
A therapy involving a radioactive iodine compound (I 131) used to treat certain tumors, such as pheochromocytomas. The radiation targets tumor cells, helping to destroy them or reveal their location for imaging.
MRI:
A non-invasive imaging technique that uses powerful magnets and radio waves to create detailed images of organs and tissues inside the body. MRI is particularly effective for soft tissues and is commonly used to evaluate the brain, spine, and joints.
Mutation:
A change in the DNA sequence at a specific gene location. Mutations can have beneficial, harmful, or neutral effects on cellular functions. The term “variant” is sometimes used interchangeably with mutation.
Neuroendocrine tumor:
A tumor arising from cells that release hormones in response to nervous system signals. These tumors may produce excessive hormones, causing a range of symptoms. They can be either benign or malignant. Examples include pheochromocytomas and medullary thyroid cancer.
Norepinephrine:
A chemical produced by the adrenal gland and nerve cells that functions as both a hormone and neurotransmitter. It is released in response to stress or low blood pressure and is also known as noradrenaline.
Nuclear medicine:
A branch of medicine that utilizes radioactive substances to diagnose and treat diseases. Radioactive materials are used with imaging technologies like PET scans to locate cancer, track its spread, or evaluate treatment effectiveness.
Nuclear medicine scan:
An imaging method where radioactive substances are introduced into the body to visualize specific tissues or cells. A scanner detects the radiation emitted by the substance to create detailed images.
Palpitation:
A sensation of rapid, irregular, or forceful heartbeat that a person can feel.
PARP Inhibitor:
A class of drugs that block the PARP enzyme, which is involved in DNA repair. In cancer treatment, these inhibitors prevent cancer cells from repairing damaged DNA, causing them to die.
Paraganglioma:
A rare tumor arising from paraganglia, clusters of nerve-associated cells. These tumors can occur near the adrenal glands or other blood vessels and nerves. Paragangliomas found in the adrenal gland are called pheochromocytomas.
PET Scan:
A positron emission tomography (PET) scan is an imaging test that produces images of your organs and tissues at work. The test uses a safe, injectable radioactive chemical called a radiotracer and a device called a PET scanner.
The scanner detects diseased cells that absorb large amounts of the radiotracer, which indicates a potential health problem.
Healthcare providers frequently use PET scans to help diagnose cancer and assess cancer treatment
Pheochromocytoma:
A tumor that develops in the adrenal gland and often leads to excessive production of adrenaline. Symptoms include high blood pressure, headaches, palpitations, and nausea. These tumors are usually non-metastatic but require treatment to prevent complications.
Plasma-free metanephrines test:
A blood test used to measure metanephrines, substances produced when adrenaline and noradrenaline break down. High levels of metanephrines can indicate the presence of pheochromocytomas or paragangliomas.
PPGL:
An acronym for pheochromocytoma and paraganglioma.
PRRT:
A therapy that uses radioactive molecules to treat neuroendocrine tumors. It is also being explored as a treatment for pancreatic and prostate cancers.
Radiology:
A field of medicine that uses imaging techniques like x-rays, MRI, and ultrasound to diagnose and treat diseases.
Somatostatin receptor scintigraphy:
A scanning procedure using a radioactive hormone called octreotide to locate tumors. The hormone binds to the tumor, and a camera detects the radioactive emissions to produce images.
Sutent:
A medication used to treat certain cancers, including advanced kidney cancer and pancreatic tumors. It works by inhibiting proteins that help cancer cells grow and preventing the development of blood vessels that supply tumors.
Twenty-four-hour urine test:
This test involves collecting all urine over a 24-hour period to measure levels of catecholamines (adrenaline and noradrenaline) and their byproducts, known as metanephrines. These substances, produced when catecholamines break down, are evaluated to detect abnormalities. Elevated levels may indicate the presence of pheochromocytoma or paraganglioma.
VHL Syndrome:
An inherited disorder that causes abnormal blood vessel growth in areas like the eyes, brain, and adrenal glands. Individuals with VHL syndrome have a higher risk of developing certain cancers.
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