There are several treatment options for pheochromocytomas/paragangliomas and treatment options are recommended on a case-by-case basis. The most common treatment of solitary pheochromocytomas remains surgical resection.
Patients with solitary tumors or multiple tumors clustered in one area of the body will often undergo surgical treatment, which involves the surgical removal of the offending tumors. Prior to surgery, patients will undergo what is known as a blockade. This is the administration of one or more medications that will help maintain the patient's blood pressure in an attempt to prevent complications while under anesthesia.
Peptide Receptor Radionuclide Therapy (PRRT)
In PRRT, a cell-targeting protein (or peptide), similar to the natural circulating hormone somatostatin, is combined with a small amount of radioactive material, or radionuclide, creating a special type of radiopharmaceutical called a radiopeptide. When injected into the patient’s bloodstream, this radiopeptide travels to and binds to neuroendocrine tumor cells, delivering a targeted high dose of radiation directly to the cancer cells. The mechanism by which this radiopeptide can target the tumor cell is the abundance (called an overexpression) of a specific type of surface receptor—a protein that extends from the cell’s surface—that binds to somatostatin.
Metaiodobenzylguanidine (MIBG) is a compound that can be combined with radioactive iodine (I-131) to deliver targeted radiation therapy. I-131 MIBG can be used to treat non resectable or metastatic pheochromocytoma/paragangliomas. This targeted radionuclide therapy is able to detect and treat these tumors because the compound MIBG is very similar to norepinephrine/noradrenaline, a neurotransmitter chemical that is taken up by certain neuroendocrine cells. I-131 MIBG is used to treat neuroendocrine tumors by selectively targeting and killing neuroendocrine tumors that take up MIBG.
Chemotherapy is mainly used for the patients in whom pheochromocytomas or paragangliomas are rapidly growing, especially when multiple-organ lesions are present and a surgical approach is not feasible. Chemotherapy cycles are normally given every 3 weeks and the doses are adjusted according to the patient's blood count and tolerance.
Radiotherapy uses high powered x-rays or radioactive beams to kill cancer cells. Radiation is most harmful to rapidly growing cells, so radiation therapy damages cancer cells more than normal cells. Specifically, radiation therapy damages the DNA of cancer cells, which prevents the cancer cells from growing and dividing.
Radiofrequency ablation is an alternative to surgical removal of a tumor and is performed by an interventional radiologist. Radiofrequency ablation involves inserting a needle into the tumor and charging the needle to essentially "cook" the tumor. This is a safe and effective tumor destruction technique. Like with surgery, tumors will undergo a blockade before radiofrequency ablation to avoid complications while under anesthesia.
Cryoablation is a minimally invasive treatment that uses extreme cold to freeze and destroy diseased tissue, including cancer cells. During cryotherapy, liquid nitrogen or argon gas is applied to diseased cells located outside or inside the body. Physicians use image-guidance techniques such as ultrasound, computed tomography (CT) or magnetic resonance (MR) to help guide these freezing substances to treatment sites located inside the body.
In cases of malignant pheochromocytomas refractory to both radiation therapy and chemotherapy, tyrosine kinase inhibitors such as Sunitinib, appear to be beneficial.