Surgical treatment usually alleviates symptoms. Once diagnosed, it is recommended to be seen by a multi-disciplinary medical team with pheo para experience. 30-40% of pheochromocytomas and paragangliomas
Paraganglioma
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites. Unlike other types of cancer, there is no test that determines benign from malignant tumors; long-term followup is therefore recommended for all individuals with paraganglioma. Approximately 50% of patients with recurrent disease experience distant metastasis. The five-year survival in the setting of metastati…
Full Answer
Is there a cure for paraganglioma?
There are currently no cures for cancerous paraganglioma. However, existing treatment options may reduce the tumors and prolong survival. All paraganglioma have the potential to become metastatic, or spread to other parts of the body.
What are the treatment options for jugulotympanic paraganglioma of Fisch Class C and D?
Surgery, radiotherapy or a combined modality for jugulotympanic paraganglioma of Fisch class C and D. Clin Otolaryngol. 2018;43(6):1566‐1572. [ PubMed] [ Google Scholar] 108.
Should I talk to my doctor about my paraganglioma symptoms?
If you’re experiencing symptoms of paraganglioma, such as high blood pressure and headaches, talk to your provider. Even though paraganglioma is rare and the likelihood of having it is low, it’s important to treat high blood pressure regardless.
What is paraganglioma?
Paragangliomas are rare tumors. They can occur at any age, but they're most often diagnosed in adults between 30 and 50. Most paragangliomas have no known cause, but some are caused by gene mutations that are passed from parents to children.
How is paraganglioma treated?
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. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients.
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%.
Can a paraganglioma be removed?
Surgery. Surgery is used to remove the paraganglioma. Even if the paraganglioma can't be removed completely, your doctor may recommend an operation to remove as much as possible. If your paraganglioma produces excess hormones, you may need to take medications before surgery to control the hormones.
Which is the most common surgical treatment option for a person with pheochromocytoma?
Surgical resection of the tumor is the treatment of choice for pheochromocytoma and usually results in cure of the hypertension.
How serious is a paraganglioma?
Paraganglioma are found in 2 out of every million people each year and is the cause of high blood pressure in less than 0.2% of people with high blood pressure. However, because paraganglioma release adrenaline in uncontrolled bursts, they can cause serious health problems like stroke, heart attacks, and even death.
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.
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.
Does malignant paraganglioma come back?
Approximately 35% to 50% of malignant paragangliomas may spread to other parts of your body (metastasize). Recurrent paraganglioma: Cancer has recurred (come back) after it has been treated. It may come back in the same place or in another part of your body.
Can paraganglioma reoccur?
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 metastatic paraganglioma?
Following the definition of the world health organization, paragangliomas (PGLs) are chromaffin cell tumors developing from the sympathetic and parasympathetic ganglia throughout the abdomen and head and neck area. A PGL arising from the adrenal gland is called pheochromocytoma (PHEO).
What is the difference between paraganglioma and pheochromocytoma?
The terms pheochromocytoma and paraganglioma are often used interchangeably because morphologically and functionally these entities are the same. Strictly speaking, however, paragangliomas that arise in the adrenal medulla are defined as pheochromocytomas, and those outside the adrenal gland are called paragangliomas.
What are 4 possible treatments for pheochromocytoma?
Medications, such as alpha blockers, beta blockers and calcium channel blockers, keep smaller veins and arteries open and relaxed. This improves blood flow and decreases blood pressure. Some of these medications may also cause your heart to beat more slowly and with less force.
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.
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 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.
Can paraganglioma be treated?
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 paraganglioma surgery, either open or laparoscopic.
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.
Symptoms and Causes
Signs and symptoms of paraganglioma happen when the tumor releases too much adrenaline or noradrenaline into your blood. However, some paraganglioma tumors don’t make extra adrenaline or noradrenaline and don’t cause symptoms (are asymptomatic). Common symptoms of paraganglioma include episodes of:
Diagnosis and Tests
Since paraganglioma is a rare tumor and is sometimes asymptomatic, it can be difficult to diagnose. Healthcare providers sometimes find paragangliomas when they order a test or procedure for another reason.
Management and Treatment
Treatment options for paraganglioma depend on several factors, including:
Prevention
Unfortunately, you can’t prevent developing a paraganglioma. However, if you’re at risk for developing a paraganglioma due to certain inherited syndromes and genes, genetic counseling can help screen for paraganglioma and potentially catch it in its early phases.
Living With
If you’ve been diagnosed with paraganglioma and experience concerning symptoms, contact your healthcare provider.
Overview
Paragangliomas are rare, neuroendocrine tumors that can involve different areas of the skull base and neck.
Symptoms
Paragangliomas arising from the carotid body or vagus nerve may present as a painless neck mass. On the other hand, because of their association with the middle ear, glomus jugulare and glomus tympanicum tumors more commonly present with pulsatile tinnitus, hearing loss, and otalgia.
Diagnosis
A patient’s history, physical examination, and imaging studies are used in the diagnosis of vestibular schwannoma. A CT or MRI with and without contrast are often used to show the location and size of these highly vascular tumors. Angiography can demonstrate the tumor’s relation to blood vessels.
Treatment
Treatment decisions are informed by the patient’s symptoms such as pulsatile tinnitus or neurologic deficits, the growth, size, and location of the tumor, whether multiple tumors are present, and the patient’s general medical condition.
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.