Table 1

Main clinical and tumour characteristics of the different cohorts

Total patients n=1167
VHL-like, n=76 patients
 Age at first diagnosis mean (min-max)45.4 (20–76)
 Multiple haemangioblastomas16 (21%)
 Haemangioblastoma with another VHL tumour28 (37%)
 One VHL tumour and family history of VHL tumour32 (42%)
Multiple VHLtumours, n=70 patients
 Age at first diagnosis mean, (min-max)55 (11–81)
 Three or more VHL tumours3 (4%)
 Two VHL tumours67 (96%)
Single VHLtumour, n=75 patients
 Age at first diagnosis mean (min-max)34.6 (11–78)
 Clear cell renal cell carcinoma3 (4%)
 Cerebral haemangioblastoma27 (36%)
 Retinal haemangioblastoma10 (13.3%)
 Other tumours35 (46.6%)
PPGL, n=946 patients
 Age at first diagnosis mean (min-max)43 (8–94)
 Benign PPGL869 (92%)
 Single benign PPGL771 (82%)
 Multiple benign PPGL98 (10%)
 Metastatic PPGL77 (8%)
 Single metastatic PPGL67 (7%)
 Multiple metastatic PPGL10 (1%)
 Familial PPGL17 (2%)
  • PPGL, paraganglioma; VHL, von Hippel-Lindau.