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MyPART - My Pediatric and Adult Rare Tumor Network
 

Pheochromocytoma

What is pheochromocytoma? 

Pheochromocytoma is a type of neuroendocrine tumor that grows from cells called chromaffin cells. These cells produce hormones needed for the body and are found in the adrenal glands. The adrenal glands are small organs located in the upper region of the abdomen on top of the kidneys. About 80-85% of pheochromocytomas grow in the inner layer of the adrenal gland, called the adrenal medulla. About 15-20% of pheochromocytomas grow outside of this area and are called extra-adrenal pheochromocytomas or paragangliomas. 

Most pheochromocytomas are benign, which means they are not cancer and do not spread to other parts of the body. Only about 10% of pheochromocytomas spread to other parts of the body.

How common is pheochromocytoma?

It is unknown how many people have pheochromocytoma because many people are never diagnosed. Most cases of pheochromocytoma occur in people aged 30 to 50 years old. One estimate suggests about only 8 people per 1 million people have pheochromocytoma, but this estimate may be low.

How is pheochromocytoma diagnosed?

Some people with pheochromocytoma have symptoms, but others don’t. Symptoms may occur as often as several times a day to a couple of times per month. Some people may feel intense symptoms that last for a short period of time, called “paroxysmal attacks”. These symptoms can include:
•    High blood pressure
•    Headaches
•    Irregular heartbeat
•    Sweating  

Lab Tests: If you have symptoms of pheochromocytoma, your doctor will order lab tests of your urine and blood to check your hormone levels.

Imaging: Your doctor will use imaging scans such as CT, MRI, and PET to look at where the tumor is and how big it is. They will also check for signs that the tumor has spread to other parts of the body.

How is pheochromocytoma treated?

Treatment of pheochromocytoma may involve many different doctors, including doctors who specialize in hormone disorders and doctors who diagnose and treat cancer. Treatment options to discuss with your doctor include: 

Medications: Your doctor may give you medications to control your symptoms, such as alpha blockers and beta blockers, which are drugs to control high blood pressure.

Surgery: Surgery is used to remove as much of the tumor as possible. In some cases, the entire adrenal gland may be removed.

Radiation therapy and chemotherapy: Radiation and chemotherapy treatments are used when pheochromocytoma has spread to other parts of the body. 

Does pheochromocytoma run in families?

In some cases, pheochromocytoma can run in families. About 25-35% of cases of pheochromocytoma may be inherited. Some of these inherited cases may be associated with a genetic condition, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis. 

How does pheochromocytoma form?

Scientists have found mutations in approximately 20 different genes that they think may lead to pheochromocytoma and paraganglioma. 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 may play a role in forming pheochromocytoma and paragangliomas. In many cases, it is not known what causes pheochromocytoma to form.

If you have pheochromocytoma, you may have other genetic conditions that increased your chance of getting pheochromocytoma. These genetic conditions include:
•    Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B)
•    Von Hippel-Lindau (VHL) syndrome
•    Neurofibromatosis type 1 (NF1)
•    Hereditary paraganglioma syndrome
•    Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST])
•    Carney triad (paraganglioma, GIST, and pulmonary chondroma)

What is the prognosis for someone with pheochromocytoma?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: 

•    Where the tumor is in your body
•    If the cancer has spread to other parts of your body
•    How much of the tumor was taken out during surgery 

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis

Doctors estimate pheochromocytoma survival rates by how groups of people with pheochromocytoma have done in the past. Patients with a small pheochromocytoma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with pheochromocytoma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.