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. 2020 Jan 29;57(11):752–759. doi: 10.1136/jmedgenet-2019-106519

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 haemangioblastomas 16 (21%)
 Haemangioblastoma with another VHL tumour 28 (37%)
 One VHL tumour and family history of VHL tumour 32 (42%)
Multiple VHL tumours, 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 VHL tumour, 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.