Treatments for cancerous pheochromocytoma

The following are treatment options for cancerous pheochromocytomas. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.

The treatments depend on whether the cancer can be completely removed with surgery (is resectable) or can’t be completely removed with surgery (is unresectable).

Doctors often describe cancerous pheochromocytomas using the words local, regional and metastatic.

Local means that the cancer is only in the adrenal gland and has not spread to other parts of the body.

Regional means that the cancer has spread close to or around the adrenal gland.

Metastatic means that the cancer has spread to a part of the body farther from the adrenal gland.

Different types of supportive therapy drugs may be offered as treatment for pheochromocytomas.

Drugs to control high blood pressure and irregular heart rhythms are also called alpha blockers and beta blockers. These drugs are usually given 1 to 2 weeks before surgery. Blood pressure and heart rhythms must be normal before surgery can be done.

Drugs to replace very low levels of adrenal gland hormones after surgery are also called hormone replacement therapy (HRT) drugs. They are given until hormone levels return to normal. Hormone levels usually return to normal within 2 weeks after surgery if the cancer was completely removed. If both of the adrenal glands are removed, you will need to take HRT for the rest of your life.

Drugs to help prevent bone loss such as bisphosphonates may be given if cancer has spread to the bones.

Side effects of supportive drugs will depend mainly on the type and dose of the drug. Tell your healthcare team if you have side effects that you think are from supportive therapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Surgery is a medical procedure to examine, remove or repair tissue. Surgery, as a treatment for cancer, means removing the tumour or cancerous tissue from your body.

Surgery is usually used to treat pheochromocytomas. Non-cancerous pheochromocytomas can usually be completely removed with surgery. The type of surgery done for cancerous pheochromocytomas depends on the spread of the cancer.

An adrenalectomy is a surgery to remove the adrenal gland. It is done if the cancer is only in the adrenal gland (called local pheochromocytoma).

Debulking means removing as much of the cancer as possible. It may be used if the cancer has spread into nearby tissues (called regional pheochromocytoma). Debulking may also be used if the cancer has spread to distant tissues (metastasis) or the tumour has come back (recurred) after treatment.

Surgery for metastasis may be used to remove cancer that has spread to the liver or the lungs.

Side effects of surgery will depend mainly on the type and site of surgery and your overall health. Tell your healthcare team if you have side effects that you think are from surgery. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Surgery for adrenal gland cancer may cause:

• pain
• less urine than normal
• changes in blood pressure
• a low blood sugar level

Find out more about surgery.

Radiation therapy uses high-energy rays or particles to destroy cancer cells.

Radiation therapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. The types of radiation therapy are:

• external radiation therapy to treat cancer that has spread to the bones
• targeted radiation therapy with radioactive metaiodobenzylguanidine (MIBG)

During radiation therapy, your healthcare team protects healthy cells in the treatment area as much as possible. Side effects of radiation therapy will depend mainly on the size of the area being treated, the specific area or organs being treated, the total dose of radiation and the treatment schedule. Tell your healthcare team if you have side effects that you think are from radiation therapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Some common side effects of radiation therapy used for adrenal gland cancer are:

• fatigue
• nausea
• loss of appetite
• diarrhea
• skin problems

Find out more about radiation therapy.

Chemotherapy uses drugs to destroy cancer cells. These drugs target rapidly dividing cells throughout the whole body. This means that chemotherapy kills cancer cells but it can also damage healthy cells.

With most types of chemotherapy, the drugs travel through the blood to reach and destroy cancer cells all over the body, including cells that may have broken away from the primary tumour. This is described as systemic therapy.

Chemotherapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. The most common combination of chemotherapy drugs used is CVD, which is cyclophosphamide, vincristine and dacarbazine.

Side effects of chemotherapy will depend mainly on the type of drug, the dose, how it's given and your overall health. Tell your healthcare team if you have side effects that you think are from chemotherapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Some common side effects of chemotherapy drugs used for adrenal gland cancer are:

• peripheral nerve damage (peripheral neuropathy)
• low blood cell counts
• nausea and vomiting
• diarrhea
• loss of appetite
• skin problems

Find out more about chemotherapy.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells and limit harm to normal cells. Targeted therapy may also be called molecular targeted therapy.

Sunitinib (Sutent) is a type of targeted therapy drug called a tyrosine kinase inhibitor (TKI). TKIs block chemicals called tyrosine kinases. These chemicals are part of the signalling process within cells. When this process is blocked, the cell stops growing and dividing.

Sunitinib may be offered to treat metastatic pheochromocytoma.

Side effects of targeted therapy will depend mainly on the type of drug, the dose, how it's given and your overall health. Tell your healthcare team if you have side effects that you think are from targeted therapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Some common side effects of sunitinib are:

• diarrhea
• fatigue
• nausea and vomiting
• taste changes
• indigestion
• sore mouth and throat
• high blood pressure (hypertension)
• liver problems, which can cause jaundice

Sunitinib may not be covered by all provincial or territorial health plans.

Find out more about targeted therapy.

Other treatments may be offered for metastatic and recurrent pheochromocytomas, including:

• radiofrequency ablation (RFA)
• transarterial chemoembolization (TACE)

Side effects will depend mainly on the type of treatment given. Tell your healthcare team if you have side effects that you think are from these treatments. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.

Watchful waiting means that your healthcare team watches the cancer closely rather than giving treatment right away. They will use tests and exams to see if the pheochromocytoma is growing or spreading. Treatment is given when you develop symptoms or the cancer changes.

This approach helps avoid side effects that can happen with treatments such as surgery or radiation therapy. There is no evidence so far that people won't live as long when they get watchful waiting compared to other treatments. And there is no evidence that watchful waiting has other negative effects if or when you start treatment.

You may be offered watchful waiting if:

• you don't have any symptoms of pheochromocytoma
• the cancer is growing very slowly and isn't affecting your health

A few clinical trials in Canada are open to people with cancerous pheochromocytoma. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

You may want to consider a type of care to make you feel better without treating the cancer itself. This may be because the cancer treatments don't work anymore, they're not likely to improve your condition or they may cause side effects that are hard to cope with. There may also be other reasons why you can't have or don't want cancer treatment.

Talk to your healthcare team. They can help you choose care and treatment for advanced cancer.