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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

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