What are the clinical manifestations of hereditary forms of pheochromocytomas?

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

In hereditary forms of pheochromocytoma, the secretory profiles vary according to the underlying syndrome. Eisenhofer et al found that pheochromocytomas associated with VHL typically produce norepinephrine only, while those associated with MEN 2 and NF1 typically produce both epinephrine and norepinephrine. Tumors in patients with germline mutations of succinate dehydrogenase subunit genes (SDHB and SDHD), which cause familial paraganglioma, principally produce dopamine. [10]


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