What exactly is a paraganglioma?
Paraganglioma is a kind of neuroendocrine tumor that develops outside of the adrenal glands near particular blood arteries and nerves.
The adrenal glands, which are positioned on top of the kidneys, are responsible for producing hormones that regulate a variety of physiological processes. Paraganglioma affects nerve cells in the peripheral nervous system, which is the component of the nervous system outside of the brain and spinal cord. Extra-adrenal pheochromocytomas are another name for these tumors.
Paragangliomas can spread to other regions of the body in 35-50 percent of cases.
What is the prevalence of paraganglioma?
Paraganglioma is a rare cancer that affects just 2 people out of every 1 million people.
It is most common in adults between the ages of 30 and 50.
Paraganglioma is diagnosed in a variety of ways.
Although some persons with paraganglioma experience symptoms, others do not. Among the signs and symptoms are:
- High blood pressure
- Rapid pulse
- Sweating
- Headache
- Tremors or shaking
Lab Tests: If you have paraganglioma symptoms, your doctor will request urine and blood tests to examine your hormone levels.
Imaging: Tests such as MRI, CT, and PET will be used by your doctor to determine the location and size of the tumor. They'll look for symptoms that the tumor has spread to other places of the body, as well.
What is the treatment for paraganglioma?
Specialists that specialize in hormone abnormalities and doctors who identify and treat neuroendocrine tumors may be involved in the treatment of paraganglioma. The following are some treatment options to consider with your doctor:
- Medications: Your doctor may prescribe medications to help you manage your symptoms, such as alpha blockers, which may be followed by beta blockers, which are blood pressure-lowering therapies.
- Keep an eye out and wait: The tumor may develop slowly in some circumstances. In this scenario, it could be safer for your doctor to monitor your tumor rather than treat it.
- Surgery: Once you've been diagnosed with paraganglioma, you may need surgery to remove the tumor. In the event that surgery is not a possibility, your doctor will explore other choices with you.
- Radiation therapy: Radiation therapy can be used to assist ease symptoms and inhibit the growth of cancers.
It's critical to consult with a team of experts to determine the best course of action for you.
Is paraganglioma a hereditary disease?
Paraganglioma can, but does not usually, run in families. Some of these hereditary instances, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis Type 1, may be linked to a genetic disorder.
What causes a paraganglioma to develop?
Pheochromocytoma and paraganglioma are thought to be caused by mutations in roughly 20 distinct genes, according to scientists. Pheochromocytoma and paraganglioma may be caused by mutations in the genes RET, VHL, NF1, SDHA, SDHB, SDHC, SDHD, SDHAF2, MDH2, IDH1, PHD1/PHD2, HIF2A/EPAS1/2, TMEM127, MAX, HRAS, MAML3, and CSDE1.
Some genetic disorders have been linked to paraganglioma, according to research. The following are examples of genetic conditions:
- Types A and B of multiple endocrine neoplasia 2 syndrome (MEN2A and MEN2B)
- Syndrome of von Hippel-Lindau (VHL)
- Type 1 neurofibromatosis (NF1)
- Hereditary paraganglioma syndrome is a kind of paraganglioma that runs in families.
- Stratakis-Carney dyad (paraganglioma and gastrointestinal stromal tumor [GIST])
- Triad of Carney (paraganglioma, GIST, and pulmonary chondroma)
What is the prognosis for paraganglioma patients?
Prognosis is a prediction of how a condition will effect you in the long run. Every person is unique, and prognosis is influenced by a variety of circumstances, including:
The location of the tumor in your body: If your cancer has spread to other regions of your body, you should seek medical help immediately.
What percentage of the tumor was removed during surgery?
It is critical to speak with your doctor if you want to learn more about your prognosis. The National Cancer Institute also provides materials to assist you understand cancer prognosis.
The five-year survival rate for patients with a minor paraganglioma that has not progressed to other regions of the body is around 95 percent. Patients with recurrent paraganglioma or paraganglioma that has spread to other regions of the body have a five-year survival rate of 34 percent to 60%.