Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review

Annika M.A. Berends, Edward Buitenwerf, Ronald R. de Krijger, Nic J.G.M. Veeger, Anouk N.A. van der Horst-Schrivers, Thera P. Links, Michiel N. Kerstens

Research output: Contribution to journalArticlepeer-review

149 Citations (Scopus)


Introduction: Recent years have seen major changes in clinical practice which may have affected the incidence rates of pheochromocytoma(PCC)/sympathetic paraganglioma(sPGL). There is, however, a lack of up-to-date information describing trends in these incidence rates. Methods: We searched the Dutch pathology registry to identify all histopathologically confirmed cases of PCC/sPGL diagnosed between 1995 and 2015. We calculated incidence rates according to age category as well as age-standardized incidence rates (ASR). We also searched Medline and Embase to find data on nationwide incidence rates of PCC/sPGL. Results: The nationwide pathology study revealed a total of 1493 patients with either PCC or sPGL. The ASR for PCC increased from 0.29 (95% CI: 0.24–0.33) to 0.46 (95% CI: 0.39–0.53) per 100,000 person-years in the periods 1995–1999 and 2011–2015, respectively. For sPGL the ASR in these same periods were 0.08 (95% CI: 0.06–0.10) and 0.11 (95% CI: 0.09–0.13) per 100,000 person-years, respectively. Concomitantly, PCC size decreased (β −0.17; P <.001) and age at diagnosis increased (β 0.13; P =.001). Our systematic search yielded 3 papers reporting on a total of 530 PCC/sPGL cases, showing a combined annual incidence rate varying from 0.04 to 0.21 per 100,000 person-years. Conclusion: Incidence rates of PCC/sPGL have increased significantly over the past two decades. This trend coincides with a higher age and a smaller tumor size at diagnosis. Most likely these observations are at least in part the result of changes in clinical practice during the study period, with a more intensified use of both imaging studies and biochemical tests for detecting PCC/sPGL.

Original languageEnglish
Pages (from-to)68-73
Number of pages6
JournalEuropean Journal of Internal Medicine
Publication statusPublished - May 2018
Externally publishedYes


  • Incidence
  • Pheochromocytoma (PCC)
  • Sympathetic paraganglioma (sPGL)


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