What is pheochromocytoma and how does it affect you?
Pheochromocytoma is a neuroendocrine tumor that develops from cells known as chromaffin cells. The adrenal glands contain cells that create hormones that the body need. The adrenal glands are tiny organs that sit on top of the kidneys in the upper abdomen. Pheochromocytomas form in the inner layer of the adrenal gland, termed the adrenal medulla, in around 80-85% of cases. Extra-adrenal pheochromocytomas, also known as paragangliomas, are pheochromocytomas that develop outside of the adrenal gland.
Pheochromocytomas are benign tumors that do not spread to other regions of the body and do not cause cancer. Pheochromocytomas only spread to other regions of the body in roughly 10% of cases.
What is the prevalence of pheochromocytoma?
Because many people are never diagnosed, it is unknown how many people with pheochromocytoma. Pheochromocytoma is most common in adults between the ages of 30 and 50. According to one estimate, only 8 persons per million have pheochromocytoma, however this figure might be underestimated.
How can you know if you have pheochromocytoma?
Pheochromocytoma can cause symptoms in some people, but not in others. Symptoms might appear anywhere from numerous times per day to a few times each month. Paroxysmal attacks are a type of symptom that occurs in certain persons and lasts for a brief amount of time. These signs and symptoms include:
- Irregular heartbeat
- Sweating
- High blood pressure
- Headaches
Lab Tests: If you have pheochromocytoma symptoms, your doctor will request urine and blood tests to examine your hormone levels.
Imaging tests such as CT, MRI, 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 pheochromocytoma?
Doctors who specialize in hormone abnormalities and doctors who detect and treat cancer may be involved in the treatment of pheochromocytoma. 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 and beta blockers, which are blood pressure medications.
Surgery: To remove as much of the tumor as feasible, surgery is utilized. The entire adrenal gland may be removed in rare circumstances.
When pheochromocytoma has spread to other regions of the body, radiation and chemotherapy therapies are performed.
Is pheochromocytoma a hereditary disease?
Pheochromocytoma can run in families in some situations. Pheochromocytoma can be inherited in 25-35 percent of instances. Some of these hereditary instances, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis, may be linked to a genetic disorder.
What causes pheochromocytoma?
Pheochromocytoma and paraganglioma are thought to be caused by mutations in roughly 20 distinct genes, according to scientists. Pheochromocytoma and paragangliomas can 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. The exact etiology of pheochromocytoma formation is unknown in many situations.
You may have additional inherited diseases that raised your risk of developing pheochromocytoma if you have pheochromocytoma. The following are examples of genetic conditions:
- Types A and B of multiple endocrine neoplasia 2 syndrome (MEN2A and MEN2B)
- VHL (von Hippel-Lindau) syndrome
- 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 a pheochromocytoma patient?
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.
Doctors calculate pheochromocytoma survival rates by looking at how previous sets of pheochromocytoma patients fared. The five-year survival percentage for patients with a small pheochromocytoma that has not migrated to other regions of the body is around 95 percent. Patients with recurrent pheochromocytoma or pheochromocytoma that has progressed to other sections of the body have a five-year survival rate of 34 percent to 60%.