Pheochromocytoma – What it is, Symptoms and Treatments of this condition. Also, a Pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare, usually non-cancerous (benign) tumor that develops in an adrenal gland . This type of tumor usually affects one of your two adrenal glands, but it can affect both.
If you have a pheochromocytoma , the tumor releases hormones that cause high or episodic blood pressure. Untreated, a pheochromocytoma can result in serious or fatal damage to other body systems, especially the cardiovascular system.
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Most people with pheochromocytoma are between 20 and 50 years old, but the tumor can develop at any age. Surgical treatment to remove a pheochromocytoma usually returns blood pressure to normal. So, check out now Pheochromocytoma – What is it, Symptoms and Treatments:
What is Pheochromocytoma: Pheochromocytoma ( PCC) is a neuroendocrine tumor of the medulla of the adrenal glands (originating from chromaffin cells), or extra-adrenal chromaffin tissue that has not managed to involute after birth, which secretes large amounts of catecholamines, in the mostly norepinephrine, more epinephrine to a lesser extent.
Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytoma ) are closely related, although less common, Tumors that originate in the ganglia of the sympathetic nervous system and are named based on the primary anatomic site of origin. The term is from Greek phaios “dark”, chroma “color”, kytos “cell”, oma “tumor”.
Causes of Pheochromocytoma: Researchers do not know what causes a Pheochromocytoma . The tumor develops into specialized cells, called chromaffin cells, located in the center of an adrenal gland . These cells release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine), which help control many bodily functions such as heart rate, blood pressure, and blood sugar.
The Role of Hormones: Adrenaline and noradrenaline are hormones that trigger your body’s fight-or-flight response to a perceived threat. Hormones require higher blood pressure, a faster heart rate, and an increase in other body systems that allow you to react quickly. A Pheochromocytoma results in the irregular and excessive release of these hormones.
Related Tumors: While most chromaffin cells reside in the adrenal glands, small groups of these cells are also in the heart, head, neck, bladder, back wall of the abdomen, and along the spine. Tumors in these chromaffin cells, called paragangliomas, can result in the same effects in the body.
Symptoms of Pheochromocytoma: Signs and symptoms of Pheochromocytoma usually include:
- High pressure
- intense sweating
- Fast heartbeat (tachycardia)
- Pale face (pallor)
- Shortness of breath (dyspnea)
Less common signs or symptoms may include:
When to See a Doctor: Pheochromocytoma signs and symptomscan be caused by a number of conditions. Therefore, it is important to get an immediate diagnosis. Although high blood pressure is a primary sign of a pheochromocytoma , most people with high blood pressure do not have an adrenal tumor. Talk to your doctor if any of the following Pheochromocytoma factors are relevant to you:
- Difficulty controlling high blood pressure with current treatment plan
- Family history of Pheochromocytoma
- Family history of a related genetic disorder: multiple endocrine neoplasia, type II (MEN II); von Hippel-Lindau disease; Familial paraganglioma or neurofibromatosis 1 (NF1)
Pheochromocytoma Risk Factors: People with rare hereditary disorders are at an increased risk of developing a pheochromocytoma or paraganglioma, and the tumors associated with these disorders are more likely to be cancerous. These genetic conditions include the following:
- Multiple endocrine neoplasia, type II (MEN II) is a disorder that results in tumors in more than one part of the body’s hormonal (endocrine) system. Other MEN II-associated tumors may appear in the thyroid, parathyroid, lips, tongue, and gastrointestinal tract.
- Von Hippel-Lindau disease can result in tumors in several locations, including the central nervous system, endocrine system, pancreas, and kidneys.
- Neurofibromatosis 1 (NF1) results in multiple skin tumors (neurofibromas), pigmented patches of skin, and optic nerve tumors.
- Hereditary paraganglioma syndromes are inherited disorders that result in pheochromocytoma or paragangliomas.
Complications of Pheochromocytoma: High blood pressure can damage multiple organs, particularly the tissues of the cardiovascular system, brain, and kidneys. Untreated high blood pressure associated with Pheochromocytoma can result in a number of critical conditions, including:
- Heart disease
- Brain stroke
- kidney failure
- acute respiratory disorder
- Damage to the nerves of the eye
Diagnosis of Pheochromocytoma: Your doctor may order a series of tests to diagnose Pheochromocytoma . These diagnostic tests for Pheochromocytoma include:
Laboratory Tests: Your doctor will likely order the following tests that measure levels of adrenaline, noradrenaline, or byproducts of these hormones in your body:
- 24 hour urine test. You will be asked to collect a urine sample every time you urinate during a 24-hour period. Ask for written instructions on how to store, label, and return samples.
- Blood test. You will draw blood for lab work. Talk to your doctor about special preparations, such as fasting or skipping medication. Don’t miss a dose of medication without your doctor’s instructions.
Imaging Tests: If the results of laboratory tests indicate the possibility of a pheochromocytoma or paraganglioma, your doctor will order one or more imaging tests to locate a possible tumor. These tests can include:
- CT scan, a specialized X-ray technology
- MRI, which uses radio waves and a magnetic field to produce detailed images
- M-iodobenzylguanidine (MIBG), a scanning technology that can detect small amounts of an injected radioactive compound absorbed by certain tumors
- Tomografia por emissão de positrons (PET), uma tecnologia de varredura que também pode detectar compostos radioativos absorvidos por um tumor
Descoberta Acidental: Um tumor em uma glândula adrenal pode ser encontrado durante estudos de imagem realizados por outros motivos. Nesses casos, o seu médico pediria testes adicionais para determinar a natureza do tumor.
Teste Genético: Seu médico pode recomendar testes genéticos para determinar se um Feocromocitoma está relacionado a uma doença hereditária. A informação sobre possíveis fatores genéticos pode ser importante por vários motivos:
- Como alguns distúrbios hereditários podem causar múltiplas condições, os resultados do teste podem indicar a necessidade de detectar outros problemas médicos.
- Because some disorders are more likely to be recurrent or cancerous (malignant), your test results can affect treatment decisions or long-term plans to monitor your health.
- Your test results may indicate that other family members should be screened for Pheochromocytoma or related conditions.
Ask your doctor about genetic counseling services that can help you understand the benefits and implications of genetic testing.
Pheochromocytoma Treatments: The primary treatment for a Pheochromocytoma is surgery to remove the tumor. Before undergoing surgery, your doctor will prescribe specific blood pressure medications that block the actions of high adrenaline hormones to lower your risk of developing dangerous blood pressure during surgery. Pheochromocytoma treatmentsinclude:
Preoperative Medications: You will likely take two medications for seven to ten days that help lower your blood pressure before surgery. These medications will replace or add to other blood pressure drugs you take.
- Alpha blockers keep small arteries and veins open and relaxed, improving blood flow and lowering blood pressure. Alpha blockers include phenoxybenzamine (Dibenzyline), doxazosin (Cardura) and prazosin (Minipress). Side effects can include irregular heartbeat, dizziness, fatigue, vision problems, sexual dysfunction in men and swelling in their limbs.
- Beta blockers cause your heart to beat slower and less forcefully. They also help keep blood vessels open and relaxed. In preparation for surgery, a beta blocker is added several days after starting the alpha blocker. Beta blockers include atenolol (Tenormin), metoprolol (Lopressor, Toprol-XL), and propranolol (Inderal, Innopran XL). Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing, and swelling in the limbs.
- High-salt diet Alpha and beta blockers widen (dilate) blood vessels, causing the amount of fluid inside the blood vessels to be low. This can cause dangerous drops in blood pressure with the position. A high-salt diet will draw more fluid into your blood vessels, preventing the development of low blood pressure during and after surgery.
Surgery: In most cases, the entire adrenal gland with a pheochromocytoma is removed with laparoscopic or minimally invasive surgery. Your surgeon will make some small openings through which he or she inserts wand devices equipped with video cameras and small tools.
The remaining healthy adrenal gland performs the functions normally performed by two, and blood pressure usually returns to normal. In some unusual situations, such as when the other adrenal gland has been removed, surgery may remove just the tumor, bypassing some healthy tissue.
If a tumor is cancerous (malignant), the effectiveness of surgery may depend on removing the tumor and all the cancerous tissue. However, even if all the cancerous tissue is not removed, surgery can limit hormone production and provide some control over blood pressure.
Cancer treatments: As cancer is rare among Pheochromocytoma cases , research on the best treatments is relatively limited. Treatments for malignant tumors and metastatic cancer related to a Pheochromocytoma may include:
- Radionuclide treatment. This radiation therapy combines MIBG, a compound that attaches to adrenal tumors, with a type of radioactive iodine. The goal of treatment is to deliver radiation therapy to a specific site and kill cancer cells.
- Chemotherapy. Chemotherapy is the use of powerful drugs that kill rapidly growing cancer cells.
- Cancer-specific therapies. These drugs inhibit the function of naturally occurring molecules, promoting the growth and spread of cancer cells.
Prevention of Pheochromocytoma: There are no known ways to prevent Pheochromocytoma .