Highlights
- •Up-to-date epidemiological data on PCC/sPGL are lacking.
- •The incidence rate of PCC/sPGL has increased significantly during the past two decades.
- •This rise in incidence rate is accompanied by a reduction in tumor size and a higher age at diagnosis.
- •The observed epidemiological changes are most likely the result of more intensified use of better diagnostics.
Abstract
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.
Keywords
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Article info
Publication history
Published online: January 18, 2018
Accepted:
January 10,
2018
Received in revised form:
January 5,
2018
Received:
November 20,
2017
Identification
Copyright
© 2018 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
