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Treatment of malignant pheochromocytomas

Malignant pheochromocytomas are divided into categories based on the extent of the tumour.

  • Localized refers to apparently benign tumours that are confined to the adrenal gland.
  • Regional refers to tumours that have extended into nearby tissues, lymph nodes or organs.
  • Metastatic refers to tumours that have spread to distant sites, such as the lymph nodes, bones, lungs or liver.

Recurrent pheochromocytoma means that the cancer that has come back (has recurred) after it has been treated.

The following are treatment options for malignant pheochromocytomas. The types of treatments given are based on the unique needs of the person with cancer.

Supportive therapy

Supportive therapy is an essential part of treatment for malignant pheochromocytoma. It is used to control high blood pressure and irregular heart rhythms caused by excessive hormones produced by the tumour. Blood pressure and heart rhythms must return to normal before the person can have surgery.

Surgery

Surgery is the primary treatment for malignant pheochromocytomas. The types of surgery done depend on the extent of the tumour:

  • localized – adrenalectomy (removal of the adrenal gland)
  • regional – adrenalectomy and surgical debulking (removal of as much of the tumour as possible)
  • metastatic or recurrent – surgical debulking

After surgery, hormone replacement therapy may be needed until hormone levels return to normal. If both adrenal glands are removed, the people will need steroid replacement for the rest of their lives.

Surgery is sometimes done to remove liver metastases or lung metastases.

Radiation therapy

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

  • external beam radiation to treat bone metastases
  • targeted radiation therapy with 131I-metaiodobenzylguanidine (131I-MIBG)

Chemotherapy

Chemotherapy may be offered to treat metastatic and recurrent malignant pheochromocytomas. The most common chemotherapy combination used is:

  • CVD
    • cyclophosphamide (Cytoxan)
    • vincristine (Oncovin)
    • dacarbazine (DTIC)

Clinical trials

People with pheochromocytoma may be offered the opportunity to participate in clinical trials. For more information, go to clinical trials.

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