Table 3

Reported prevalences and incidences

First author (year of publication)Prevalence/incidencePatients with manifest disease/asymptomatic mutation carriers/combined/unclearPrevalence; genetic screening independent of presence of malignant paraganglioma (yes/unclear)Incidence; free of malignant paraganglioma at baseline (yes/no/unclear)
Astrom (2003)45PrevalenceCombinedSDHD: 7%*
–
Unclear		n.a.
Benn (2006)14BothCombinedSDHB: 22% (manifest disease)†
SDHD: 0% (manifest disease)†
–
Unclear		SDHB: 19%*‡
SDHD: 8%*
–
Yes
Boedeker (2007)46PrevalenceManifest diseaseSDHB: 54%
SDHD: 0%
–
Unclear		n.a.
Hensen (2010)47PrevalenceCombinedSDHD: 3%*
–
Unclear		n.a.
Hensen (2011)12PrevalenceManifest diseaseSDHB: 0%
SDHD: 2%
–
Unclear		n.a.
Mannelli (2009)48PrevalenceManifest diseaseSDHB: 21%
SDHD: 4%
–
Unclear		n.a.
Neumann (2004)15PrevalenceCombinedSDHB: 34%*
SDHD: 0%*
–
Unclear		n.a.
Papaspyrou (2011)17PrevalenceManifest diseaseSDHB: 20%
SDHD: 23%
–
Unclear		n.a.
Peczkowska (2008)49PrevalenceManifest diseaseSDHD: 6%
–
Unclear		n.a.
Ricketts (2010)50PrevalenceCombinedSDHB: 14%*
SDHD: 3%*
–
Unclear		n.a.
Solis (2009)51PrevalenceCombinedSDHB: 3%*§
–
Yes		n.a.
Srirangaligam (2008)52IncidenceCombinedn.a.SDHB: 16%*
–
Unclear

  • *Data not separately reported for patients with manifest disease and asymptomatic mutation carriers.

  • †Prevalence calculated on the basis of reported numbers of patients, in whom metastases were identified at first surgery. Therefore, we assume this reported prevalence concerns only patients with manifest disease.

  • ‡Incidence calculated on the basis of reported numbers of patients with malignant paraganglioma at follow-up, excluding the number of patients (11) already diagnosed with malignant paraganglioma at first surgery.

  • §The authors screened 40 relatives of a proband with metastatic paraganglioma for malignant disease. To avoid an invalid risk estimate due to selection bias, we excluded the proband for our analyses.

  • n.a., not applicable; n.r., not reported.