Many paragangliomas do not require immediate treatment. Active Surveillance If you have a small tumor that isn’t causing symptoms, your doctor may recommend periodic MRI imaging and physical exams without other interventions. Radiation Therapy A form of radiation therapy called stereotactic radio surgery or CyberKnife may be recommended.
What is the treatment for hormone producing paraganglioma?
In people with hormone-producing paragangliomas, it's critical to control blood pressure before surgery or other therapy. Surgery. Surgery to remove the tumor is generally the first choice for treatment, if feasible. Even if the tumor has spread, surgery is often used to reduce its size.
Is paraganglioma in the head and neck curable?
Paraganglioma in the head and neck are less likely to be metastatic than tumors in the chest, abdomen and pelvis. Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. If possible, the treatment of choice for the condition is surgery to remove the tumor (s), but there are other treatment options.
What are the symptoms of paraganglioma?
Paraganglioma cells commonly secrete hormones known as catecholamines or adrenaline, which is the fight-or-flight hormone. This can cause episodes of high blood pressure, a rapid heartbeat, sweating, headache and tremors. Paraganglioma treatment most often involves surgery to remove the tumor.
How is embolization used to treat paraganglioma?
At Mount Sinai, we use embolization to treat paragangliomas is embolization. This state-of-the-art technique can shrink the tumor and reduce bleeding, which makes it much easier to remove surgically.
How long can you live with paraganglioma?
Patients with a small paraganglioma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with paraganglioma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.
Are all paragangliomas cancer?
Paragangliomas are usually noncancerous (benign). But some paragangliomas can become cancerous (malignant) and spread (metastasize) to other parts of the body. Paragangliomas are rare tumors. They can occur at any age, but they're most often diagnosed in adults between 30 and 50.
Should you biopsy a paraganglioma?
However, a tumor suspected to be a pheochromocytoma or paraganglioma should not have a biopsy, unless it is absolutely necessary to confirm a diagnosis. A biopsy of these tumors can cause a serious and even life-threatening release of catecholamines.
How long does it take for a paraganglioma to grow?
14, 17 The variation in the growth rate of growing paragangliomas is remarkable; we estimated a Td between 0.6–21.5 years. This observation was made in malignant tumors as well and can be explained in part by the influence of tumor size on growth rate.
Is paraganglioma curable?
There are currently no cures for cancerous paraganglioma. However, existing treatment options may reduce the tumors and prolong survival.
What type of cancer is paraganglioma?
A paraganglioma is a rare but treatable neuroendocrine tumor that usually forms along major blood vessels and nerve pathways in your neck and head. In most cases, the tumor is benign, but it can be malignant (cancer).
Is a paraganglioma a brain Tumour?
What is a paraganglioma? A paraganglioma is a type of tumour that arises from the peripheral nervous system (part of the nervous system outside the brain and spinal cord), which is further divided into the sympathetic nervous system and the parasympathetic nervous system.
Is paraganglioma a sarcoma?
Genetic Disease. Paraganglioma and gastric stromal sarcoma is a genetic disease, which means that it is caused by one or more genes not working correctly. Questions: What is a gene?
Can you feel a paraganglioma?
These tumors usually appears in the neck, but can extend into the space between the head and the neck. Some of most common symptoms are pain, hoarseness, fainting (syncope), difficulty swallowing (dysphagia), eye problems (Horner syndrome), and paralysis of the tongue.
Do paragangliomas come back?
This is called follow-up care. People treated for a pheochromocytoma or paraganglioma need lifelong follow-up care. This is important because pheochromocytomas and paragangliomas can recur years after initial treatment. This care may include regular physical examinations, medical tests, or both.
What is paraganglioma syndrome?
Hereditary paraganglioma-pheochromocytoma syndrome is a condition in which tumors develop in structures called paraganglia. Paraganglia are bundles of cells of the peripheral nervous system (the nerves outside the brain and spinal cord). A tumor that develops in the paraganglia is called a paraganglioma.
How old do you have to be to get paraganglioma?
Paraganglioma can occur at any age, but most commonly affects people between the ages of 20 and 50. While very rare, the illness often causes a range of symptoms that when recognized can help with diagnosis. Many of these symptoms can be caused by multiple other conditions as well.
What happens to the blood after paraganglioma surgery?
After surgery, the blood or urine are checked to ensure metanephrine levels and subsequently, catecholamine levels have returned to normal. Normal metanephrine levels are a sign that all the paraganglioma cells were removed, if the tumor was secreting catecholamines.
What is the treatment for pheo para?
If possible, the treatment of choice for the condition is surgery to remove the tumor (s), but there are other treatment options. Surgical treatment usually alleviates symptoms. Once diagnosed, it is recommended to be seen by a multi-disciplinary medical team with pheo para experience.
How long after paraganglioma surgery should you have a blood test?
Urine or plasma tests should be repeated 2-8 weeks after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients. Yearly urine or plasma tests for paraganglioma should be performed for life to detect remaining disease, return of the disease, or the development of metastases. For most people, follow-up CT or MRI is not needed if urine and plasma test results are normal. Exceptions to this may include the identification of a genetic mutation or if the primary tumor was large. Read more about genetic mutations on this page.
Where are paragangliomas found?
Approximately 85% of paragangliomas are in the abdomen, 12% are found in the chest, and only about 3% are in the head and neck. This figure shows where paraganglioma can be found. Paraganglioma in the head and neck are less likely to be metastatic than tumors in the chest, abdomen and pelvis.
What hormones are involved in paraganglioma?
Just like pheochromocytoma, paraganglioma is also characterized by the presence of an excess amount of hormones called catecholamines, which include norepinephrine (noradrenaline), epinephrine (adrenaline), and dopamine. It is these hormones that lead to persistent or episodic high blood pressure and other symptoms.
Is paraganglioma a slow growing tumor?
Paraganglioma (păr′ə-găng′glē-ō′mə) is a rare, slow-growing tumor that is closely related to pheochromocytoma. It originates in the parasympathetic or sympathetic nervous system. They occur in both men and women equally, and they affect every race of people.
What is the best treatment for paraganglioma?
Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays and protons, to control tumor growth. Radiation therapy might be recommended if the paraganglioma can't be removed completely with surgery. It can also be used to relieve pain caused by a paraganglioma that spreads to other parts of the body.
What to do if paraganglioma is not removed?
Usually this is done with medications. If the paraganglioma can't be removed completely during an operation or if it spreads to other parts of the body, additional treatments may be considered. Treatment options include: Treatments to control hormones produced by the tumor.
How to diagnose paraganglioma?
Tests used to diagnosed paraganglioma include: 1 Blood and urine tests. Your doctor may recommend blood tests and urine tests to measure the levels of hormones in your body. These tests may detect extra hormones produced by paraganglioma cells or a tumor marker called chromogranin A. 2 Imaging tests. Imaging tests create pictures of the paraganglioma to help your doctor better understand the extent of the tumor and help determine the best treatment options. Tests may include MRI, CT, and specialized nuclear medicine imaging such as a metaiodobenzylguanidine (MIBG) scan and a positron emission tomography (PET) scan. 3 Genetic testing. Paragangliomas are sometimes caused by gene mutations that can be passed from parents to children. Genetic testing may be recommended as part of your care.
What hormones are secreted by paraganglioma cells?
Paraganglioma cells commonly secrete hormones known as catecholamines or adrenaline , which is the fight-or-flight hormone. This can cause episodes of high blood pressure, a rapid heartbeat, sweating, headache and tremors.
What tests are used to diagnose paraganglioma?
Tests used to diagnosed paraganglioma include: Blood and urine tests. Your doctor may recommend blood tests and urine tests to measure the levels of hormones in your body. These tests may detect extra hormones produced by paraganglioma cells or a tumor marker called chromogranin A. Imaging tests.
What tests are done to determine the extent of a paraganglioma?
Imaging tests. Imaging tests create pictures of the paraganglioma to help your doctor better understand the extent of the tumor and help determine the best treatment options. Tests may include MRI, CT, and specialized nuclear medicine imaging such as a metaiodobenzylguanidine (MIBG) scan and a positron emission tomography (PET) scan.
How to shrink a tumor in a paraganglioma?
Chemotherapy. Chemotherapy uses drugs to destroy fast-growing cells, such as cancer cells. If your paraganglioma has spread, your doctor may recommend chemotherapy to help shrink the tumors. If your paraganglioma produces excess hormones, you'll receive medications to control the hormone levels before undergoing chemotherapy.
How to diagnose paragangliomas?
Imaging scans can help us diagnose paragangliomas of all types. At Mount Sinai, we typically use these diagnostic test: 1 Computed tomography scans helps us see whether the tumor has moved into any of the nearby bony structures including the petrous bone, temporal bone, and bones of the head and neck. 2 Magnetic resonance imaging is the best diagnostic test for paragangliomas. It helps us see how big they are and whether the blood vessels have moved into adjacent structures and other vascular areas. 3 Angiography demonstrates the vascularity of paragangliomas, venous drainage, and movement into vascular structures such as the jugular vein or the sigmoid sinus.
What is the best test for paragangliomas?
Magnetic resonance imaging is the best diagnostic test for paragangliomas. It helps us see how big they are and whether the blood vessels have moved into adjacent structures and other vascular areas.
What nerves can cause paralysis?
Vagal paragangliomas can cause paralysis to the lower cranial nerves (including the 10th cranial nerve, which can cause paralysis of the diaphragm), 12th cranial nerve paralysis (with paralysis of the tongue), problems in swallowing, and problems with raising your shoulder.
How do you know if you have a caroid paraganglioma?
Caroid paragangliomas appear with an expanding and pulsing mass. When this paraganglioma appears as a simple, isolated lesion, you may experience relatively few symptoms. These tumors usually appears in the neck, but can extend into the space between the head and the neck. Some of most common symptoms are pain, hoarseness, fainting (syncope), difficulty swallowing (dysphagia), eye problems (Horner syndrome), and paralysis of the tongue.
What is a slow growing tumor that can cause palsy and spread into the skull base?
Cervical paragangliomas are slow-growing tumors that can cause palsy and spread into the skull base. It can be difficult to distinguish between tumors that affect the vagus and those that involve the carotid artery.
Can paragangliomas cause headaches?
Secretory paragangliomas can occur in up to one in eight patients and may involve the release of catecholamines. The symptoms may include headaches, hypertension or high blood pressure, palpitations, excessive sweating, and anxiety. Treatment may be difficult because it will jar the paraganglioma to release some hormones.
Is paraganglioma a tumor?
They are rarely if ever cancerous. Almost half of all paragangliomas appear in the bone on the side of the head/skull (temporal bone). These tumors are relatively simple and frequently associated with other neurological tumors that develop in embryos, such as chemodectomas and neurocristopathic tumors. At Mount Sinai, we take a multispecialty ...
What is the difference between paraganglioma and pheochromocytoma?
Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue. Paragangliomas form in nerve tissue in the adrenal glands and near certain blood vessels and nerves. Paragangliomas that form in the adrenal glands are called pheochromocytomas. Paragangliomas that form outside the adrenal glands are called extra-adrenal ...
How to treat benign pheochromocytoma?
Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed.
What is the term for paragangliomas that form outside the adrenal glands?
Paragangliomas that form outside the adrenal glands are called extra-adrenal paragangliomas. In this summary, extra-adrenal paragangliomas are called paragangliomas. Pheochromocytomas and paragangliomas may be benign (not cancer) or malignant (cancer).
What is a follow up test for pheochromocytoma?
These tests are sometimes called follow-up tests. For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. Catecholamine levels that are higher than normal can be a sign that the cancer has come back.
What is the purpose of a pheochromocytoma test?
After pheochromocytoma and paraganglioma have been diagnosed, tests are done to find out if the tumor has spread to other parts of the body.
What are the symptoms of pheochromocytoma?
Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumors are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason.
Can pheochromocytoma be detected?
Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events. Tests that examine the blood and urine are used to detect (find) and diagnose pheochromocytoma and paraganglioma.
WHAT WE OFFER YOU FOR PARAGANGLIOMAS
Globally-recognized expertise in the diagnosis and treatment of paragangliomas.
Treatment Options for Paranglioma
Recommendations for treatment depend on the size and location of the tumor, its rate of growth, and details of your overall health. Many paragangliomas do not require immediate treatment.
Clinical Trials for Paragangliomas
Clinical trials evaluate new approaches, devices, or medications in the treatment of paragangliomas. Ask your doctor or clinical trials coordinator about available trials that may be additional options for your care.
What is malignant paraganglioma?
Paraganglioma is an abnormal, rapid cell growth that originates from chromaffin cells. Chromaffin cells are nerve cells that release neurohormones found throughout the body and help carry out important body functions such as regulating blood pressure. Malignant paraganglioma is cancerous and can spread to other distant parts of the body.
Disease Causes
There is no well-known cause; however certain hereditary and genetic mutations are thought to be responsible for the development of malignant paraganglioma.
Disease Epidemiology
Malignant paragangliomas are rare forms of cancer and can occur at any age, but the peak incidence of tumor development occurs in 30-50 years.
Signs and symptoms
The patient may suffer from the following signs and symptoms. These are
Diagnosis
The following diagnostic techniques and procedures can be used to make a diagnosis. These are
Treatment
Medications to treat excess production of the neuro-hormones, surgical removal of the tumor or affected part are the main treatment options. Other treatment options are radiation therapy and chemotherapy.
What support can we give for Malignant Paraganglioma?
Malignant Paraganglioma is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer.
When To Consult Your Doctor For Paraganglioma?
You should consult your doctor when you experience certain symptoms, which are similar to those that occur in the case of Paraganglioma tumor. Persistent high blood pressure or its various episodes, which are difficult to control is the common symptom of Paraganglioma. You may experience a few of the additional problems with uncontrollable hypertension if you have Paraganglioma, such as rapid heartbeat rate, headache, and heavy sweating issue. Along with this, the tumor comes with other possible symptoms, which include the following-
What are the symptoms of paraganglioma?
Stomach or chest pain. High blood glucose or sugar levels. Symptoms in Paraganglioma patients take place whenever the tumor releases adrenaline hormones in excessive amounts in their bloodstream. A few people even experience such symptoms multiple times in one day, while others have on a weekly or monthly basis.
