People with rare inherited disorders have an increased risk of developing a pheochromocytoma or paraganglioma, and tumors associated with these disorders are more likely to be cancerous. These genetic conditions include the following:

  • Multiple endocrine neoplasia, type II (MEN II) is a disorder resulting in tumors in more than one part of the body's hormone-producing (endocrine) system. The locations of other tumors associated with MEN II include 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.
May. 02, 2014

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