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Paraganglioma

MEDLINE

Pheochromocytoma

Age groups

Incidence (geometry)

Medicine

European Journal of Internal Medicine

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.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.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.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.

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