Risk factors

People who have certain rare inherited disorders have an increased risk of pheochromocytoma or paraganglioma. Tumors associated with these disorders are more likely to be cancerous. These genetic conditions include:

• Multiple endocrine neoplasia, type 2 (MEN 2) is a disorder that results in tumors in more than one part of the body's hormone-producing (endocrine) system. Other tumors associated with this condition can appear on the thyroid, parathyroid, lips, tongue and gastrointestinal tract.
• Von Hippel-Lindau disease can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
• Neurofibromatosis 1 (NF1) results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
• Hereditary paraganglioma syndromes are inherited disorders that result in either pheochromocytomas or paragangliomas.