Pheochromocytoma: Staging and Treatment

What is staging for cancer?

Staging helps guide your treatment by telling you how much cancer is in your body and where it is. CT, PET, or MRI scans are often used to stage and diagnose pheochromocytoma (which targets the adrenal glands). A meta-iodo-benzylguanidine (MIBG) scan may also be used if your provider thinks you may have pheochromocytoma.

The standard staging system for pheochromocytoma is the "TNM system,” as described by the American Joint Committee on Cancer. The TNM system is used to describe many types of cancers. It has three parts:

• T-describes the size/location/extent of the "primary" tumor in the adrenal gland.
• N-describes if the cancer has spread to the lymph nodes.
• M-describes if the cancer has spread to other organs (metastases).

How is pheochromocytoma staged?

There are two ways to stage pheochromocytoma. The general staging system describes if the cancer is:

• Localized: The tumor is found at an early stage in the adrenal medulla or in 1 or both adrenal glands.
• Regional: Cancer has spread to surrounding tissues and/or lymph nodes.
• Metastatic: Cancer has spread to a distant part of the body.

The second, more complex staging system uses the TNM system and is listed below. Talk to your provider about your stage.

• Stage I (T1, N0, M0): The tumor is 5 cm (centimeters) wide or smaller and is only in the adrenal gland (T1). There is no spread to other organs (N0, M0).
• Stage II (T2, N0, M0): The tumor is larger than 5 cm but is only in the adrenal gland (T2). There is no spread to lymph nodes (N0) or distant organs (M0). Or it could be a symptomatic paraganglioma (a tumor that can grow in the chest, neck, and at the bottom of the skull). Most paragangliomas are benign (noncancerous) but are at risk of turning into cancer.
• Stage III (T1-T3, N1, M0): This stage can have any of the following: The tumor is 5 cm or less, it is only in the adrenal gland, and only the regional lymph nodes are affected (T1, N1, M0). The tumor is 5 cm or more and it is only in the adrenal gland, or there is a symptomatic paraganglioma, and the regional lymph nodes are affected (T2, N1, M0).
• Stage IV (any T, any N, M1): The tumor has metastasized.

How is pheochromocytoma treated?

There are different ways to treat your pheochromocytoma depending on if it is cancerous, has metastasized, and your overall health. There is not much research about treatments, but many options are available. Ask your provider what options are best for you. Your treatment options may be some of these:

• Surgery.
• Radiation Therapy, MIBG, and PRRT.
• Targeted Therapy.
• Chemotherapy.
• Clinical Trials.

Surgery

Surgery is often used whether the tumor is benign (non-cancerous) or malignant (cancerous). Surgery for pheochromocytoma may mean taking out the whole tumor and adrenal gland or the tumor and only part of the adrenal gland. This is called an adrenalectomy. If the cancer has spread to other parts of the body, those organs and tissues may also be taken out.

After the tumor is taken out, symptoms often go away. Medication is used to treat hypertension (high blood pressure) before surgery and to prevent any issues during the surgery. If both adrenal glands are removed, you will likely need to take medications to replace the hormones no longer being made by your adrenal glands.

Radiation Therapy

Radiation therapy uses high-energy x-rays to kill cancer cells. External beam radiotherapy can be used for patients who have malignant pheochromocytoma that has spread to the bone. The goal is to ease symptoms such as pain. The dose and number of treatments you get will be based on the treatment site and other factors.

Meta-iodo-benzylguanidine (MIBG), can be used to treat pheochromocytoma. MIBG has small amounts of radioactive material in it, which collect in the tumor, causing it to “light up” on a scan. Because radiation is emitted (given off) during and after the treatment, you will have to stay away from others for a few days until radiation levels have dropped to the point where it is safe for others.

Peptide receptor radionuclide therapy (PRRT) combines a medication (Lutetium 177 (Lu-177)) that targets cancer cells with a radioactive substance. This delivers radiation directly to the cancer cells. Often, you can go home after this treatment and do not need to stay away from others.

Targeted Therapy

Targeted therapies stop or slow the growth of a tumor by stopping a specific molecule needed for cancer growth. Molecular-targeted therapies, such as sunitinib, can be used after surgery if there is a high risk of cancer coming back (called recurrence).

Chemotherapy

Chemotherapy are medications that are often given intravenously (IV, into a vein) or in pill form. Chemotherapy travels through the bloodstream and throughout the body to kill cancer cells. For pheochromocytoma, chemotherapy is not used as a primary treatment, but as an option for malignant pheochromocytoma that causes symptoms and that has not responded to MIBG therapy.

The most often used chemotherapy regimen is cyclophosphamide, vincristine, and dacarbazine. Another medication that can be used is temodar. Treatment for hypertension (high blood pressure) is important if you are having chemotherapy for pheochromocytoma. Talk to your provider about ways you can manage hypertension.

Clinical Trials

You may be offered a clinical trial as part of your treatment plan. To find out more about current clinical trials, visit the Oncolink Clinical Trial Matching Service.

Making Treatment Decisions

Deciding on your treatment plan is your decision and your care team will help you. This can be overwhelming as you may be given a few options to choose from. It feels like an emergency, but you can take a few weeks to meet with different providers and think about your options and what is best for you. Friends and family can help you talk through the options and the pros and cons of each, but they cannot make the decision for you. You need to be comfortable with your decision – this will help you move on to the next steps. If you ever have any questions or concerns, be sure to call your team.

You can read more about pheochromocytoma at OncoLink.org.