What is the prevalence of pheochromocytomas?

Updated: Aug 10, 2018
  • Author: Michael A Blake, MBBCh, MRCPI, FRCR; Chief Editor: George T Griffing, MD  more...
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Pheochromocytomas are rare, reportedly occurring in 0.05–0.2% of hypertensive individuals. This accounts for only a portion of cases, however, as patients may be completely asymptomatic. A retrospective study from the Mayo Clinic revealed that in 50% of cases of pheochromocytoma, the diagnosis was made at autopsy. [21]  Approximately 10% of pheochromocytomas are discovered incidentally. [22]

A Dutch study, by Berends et al, found an increase in the age-standardized incidence rate (ASR) of pheochromocytomas and sympathetic paragangliomas in the Netherlands between 1995 and 2015. The investigators reported that the ASR between 1995 and 1999 was 0.29 per 100,000 person-years, compared with 0.46 per 100,000 person-years between 2011 and 2015. The ASRs for sympathetic paragangliomas rose between these same two periods from 0.08 to 0.11 per 100,000 person-years. There was also a trend during this 20-year period towards patients being older and tumor size smaller at diagnosis. The investigators suggested that clinical practice changes, along with greater use of imaging and biochemical studies, were at least partially responsible for the incidence increases. [23]

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