What does the locations of pheochromocytomas vary among hereditary etiologies?

Updated: Aug 10, 2018
  • Author: Michael A Blake, MBBCh, MRCPI, FRCR; Chief Editor: George T Griffing, MD  more...
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Answer

In MEN 2, VHL, and NF1, pheochromocytomas are almost always in the adrenal gland, whereas SDHB -related tumors are found in extra-adrenal sites. Patients with MEN 2, VHL, and NF1 often have a positive family history, but currently only 10% of patients with SDHB mutations have a positive family history for pheochromocytoma or paraganglioma. [38]


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