Table 1.
Incidence, age of onset, and progression of von Hippel-Lindau disease-related lesions
| Lesion | Number of Patients (%) | Mean Age of Appearance, y | Median Age of Appearance, y | SD of Appearance, y | Interval Appearance–1st Evolution, y |
Interval 1st–2nd Evolution, y |
Interval 2nd–3rd Evolution, y |
Interval 3rd–4th Evolution, y |
||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Number of Cases | Median | Number of Cases | Median | Number of Cases | Median | Number of Cases | Median | |||||
| Cerebellar hemangioblastomas | 77 (60) | 30 | 27 | 11.77 | 49 | 3.5 | 28 | 3.5 | 14 | 2 | 9 | 3 |
| Brainstem hemangioblastomas | 21 (16) | 30 | 28 | 10.87 | 10 | 7 | 5 | 1.5 | ||||
| Spinal hemangioblastomas | 76 (59) | 32 | 29 | 12.01 | 26 | 5 | 13 | 2 | 5 | 1 | ||
| Supratentorial hemangioblastomas | 15 (12) | 32 | 30 | 9.65 | 2 | 3 | ||||||
| Cystic renal lesions | 54 (42) | 37 | 32 | 12.13 | 32 | 7 | 12 | 1.5 | 4 | 4 | ||
| Renal cell carcinomas | 79 (62) | 34 | 31 | 11.39 | 22 | 8 | 12 | 1.5 | 2 | 1 | ||
| Cystic pancreatic lesions | 74 (58) | 33 | 30 | 10.91 | 36 | 5 | 14 | 3 | 4 | 3 | ||
| Pancreatic endocrine tumors | 26 (20) | 34 | 32 | 10.29 | 6 | 9 | ||||||
| Pheochromocytomas | 39 (30) | 29 | 29 | 15.49 | 5 | 9 | ||||||
| Retinal hemangiomas | 74 (58) | 29 | 25 | 14.20 | 29 | 7 | 4 | 4 | ||||
| Hypoacusia | 14 (11) | 42 | 44 | 11.5 | 4 | 2 | ||||||
| Endolymphatic sac tumors | 3 (2) | 28 | 24 | 8.33 | 3 | 2.8 | ||||||
| Epididymal cystadenomas | 12 (9) | 26 | 24 | 10.27 | 4 | 6 | ||||||
| Ovarian cysts | 11 (8) | 28 | 27 | 8.35 | ||||||||
Abbreviations: SD, standard deviation; y, years.