Table 1.
Sporadic | Hereditary | |
---|---|---|
Proportion of total cases (%) | ~70 | ~30 |
Mean age of onset (yr) | 47 | 29 |
Sex (M:F) | 1–1.25:1 | 1–1.25:1 |
No. of tumors | Single | Multiple |
Localization | Cerebellum most common | Higher proportion of extracerebellar sites (e.g. spine) |
Molecular alterations | Somatic loss of VHL detected in subset of cases | VHL loss of function with germline mutation and somatic alteration |
VHL, von Hippel-Lindau.