Total patients n=1167 | |
VHL-like, n=76 patients | |
Age at first diagnosis mean (min-max) | 45.4 (20–76) |
Multiple haemangioblastomas | 16 (21%) |
Haemangioblastoma with another VHL tumour | 28 (37%) |
One VHL tumour and family history of VHL tumour | 32 (42%) |
Multiple VHLtumours, n=70 patients | |
Age at first diagnosis mean, (min-max) | 55 (11–81) |
Three or more VHL tumours | 3 (4%) |
Two VHL tumours | 67 (96%) |
Single VHLtumour, n=75 patients | |
Age at first diagnosis mean (min-max) | 34.6 (11–78) |
Clear cell renal cell carcinoma | 3 (4%) |
Cerebral haemangioblastoma | 27 (36%) |
Retinal haemangioblastoma | 10 (13.3%) |
Other tumours | 35 (46.6%) |
PPGL, n=946 patients | |
Age at first diagnosis mean (min-max) | 43 (8–94) |
Benign PPGL | 869 (92%) |
Single benign PPGL | 771 (82%) |
Multiple benign PPGL | 98 (10%) |
Metastatic PPGL | 77 (8%) |
Single metastatic PPGL | 67 (7%) |
Multiple metastatic PPGL | 10 (1%) |
Familial PPGL | 17 (2%) |
PPGL, paraganglioma; VHL, von Hippel-Lindau.