pISSN 2234-3806eISSN 2234-3814
Table. 1.
Table. 1.
Clinical characteristics of VHL disease
| Frequency in patients, % (N) | Median (range) age at onset, yr | Surveillance leading to new manifestation, % (N) | Frequency of first onset, % (N) | Median (range) age at onset of first manifestation, yr | Symptomatic manifestation leading to first surveillance, % (N) | ||
|---|---|---|---|---|---|---|---|
| CNS | R-HB | 52 (26) | 28 (14–59) | 46.2 (12) | 18 (9) | 23 (14–42) | 85.7 (7/9) |
| ELST | 6 (3) | 20 (16–24) | 66.7 (2) | 0 (0) | NA | 0 (0) | |
| CS-HB | 76 (38) | 25 (14–54) | 60.5 (23) | 46 (23) | 24 (16–43) | 69.6 (16/23) | |
| Visceral | RCC (26) or cysts (10) | 72 (36) | 28 (18–54) | 94.5 (34) | 18 (9) | 29 (18–54) | 0 (0) |
| PCC | 30 (15) | 29 (14–54) | 80.0 (12) | 12 (6) | 24 (16–52) | 16.7 (1/6) | |
| PNET(4) or cyst (17) | 42 (21) | 25 (19–42) | 90.5 (19) | 4 (2) | 28 (26–30) | 0 (0) | |
| Epididymal cystadenoma | 6 (3) | 36 (33–38) | 0 (0) | 2 (1) | 38 | 100 (1/1) | |
| Total | 33 (14–59) | 100 (50) | 28 (14–54) | 50 (25) |
Abbreviations: VHL, von Hippel–Lindau; CNS, central nervous system; R-HB, retinal hemangioblastoma; ELST, endolymphatic sac tumor; CS-HB, craniospinal hemangioblastoma; RCC, renal cell carcinoma; PCC, pheochromocytoma; PNET, pancreatic neuroendocrine tumor; NA, not applicable.
Ann Lab Med 2022;42:352~357 https://doi.org/10.3343/alm.2022.42.3.352
© Ann Lab Med