Table 2.
Clinical manifestation in 19 patients with von Hippel–Lindau disease in Nova Scotia (actual % plus published ranges)2,5,8,10
| % of patients
|
Mean age of NS patients at first diagnosis, yr (range) | |||
|---|---|---|---|---|
| Manifestation | NS | Literature | ||
| Cerebellar hemangioblastoma | 68.4 | 44–72 | 32.0 | (10–46) |
| Spinal hemangioblastoma | 31.6 | 13–50 | 26.0 | (16–48) |
| Brainstem hemangioblastoma | 5.2 | 10–25 | 16.0 | — |
| Retinal angioma | 47.4 | 25–60 | 29.0 | (11–36) |
| Renal cell carcinoma | 31.6 | 17–45 | 34.0 | (22–41) |
| Renal cyst | 47.4 | 50–70 | 31.5 | (22–50) |
| Pheochromocytoma | 10.5 | 0–19 | 33.5 | (33–34) |
| ELST | 0.0 | 11 | ||
| Pancreatic cyst | 21.0 | 14–70 | 40.0 | (19–56) |
| Broad ligament/epididymis cysts | 5.2 | 17–54 | 33.0 | — |
ELST = endolymphatic sac tumour.