Table 3.

Existing paradigms for vHL tumor surveillance

TumorVHL Alliance 2015aBinderup et al. 2013b (Denmark)Hes et al. 2001c (the Netherlands)Kruizinga et al. 2014d
Retinal HBAnnual eye exam ≥1 yAnnual eye exam ≥0 yAnnual eye exam ≥5 yBiennial eye exam ≥7 y
PHEOAnnual PFM or UFM ≥5 yAnnual PFM ≥5 yAnnual PFM and UFM ≥10 y (serum and 24-h urine)Every 4 y screen ≥0 y
Annual abd U/S 8–15 yQ 1 y abd U/S 10–14 y
Annual abd imaging ≥16 y (alternate U/S and MRI)Q 1 y abd imaging ≥15 y (alternate U/S and MRI)
ELSTQ 2–3 y audiology eval 5–15 yQ 1 y audiology eval ≥5 yNone specifiedNone specified
Q 2 y audiology eval ≥16 y
CNS HBBiennial MRI b/s ≥16 yMRI b/s once 8–14 yBiennial MRI b/s ≥15 yAnnual MRI b/s ≥14 y
Biennial MRI b/s ≥15 y
RCCAnnual abd U/S 8–15 yAnnual abd imaging ≥15 y (U/S or MRI)Annual abd U/S 10–14 yAnnual screen ≥18 y
Annual abd imaging ≥16 y (alternate U/S and MRI)Annual abd imaging ≥15 y (alternate U/S and MRI)
Pancreatic NETAnnual abd U/S 8–15 yAnnual abd imaging ≥15 y (U/S or MRI)Annual abd U/S 10–14 yBiennial screen ≥16 y
Annual abd imaging ≥16 y (alternate U/S and MRI)Annual abd imaging ≥15 y (alternate U/S and MRI)
  • Abbreviations: abd, abdominal; eval, evaluation; h, hour; HB, hemangioblastoma; MRI b/s = MRI brain/spine; PFM, plasma-free metanephrines; Q, every; UFM, urinary fractionated metanephrines; U/S, ultrasound; y, year/years.

  • aReference 23.

  • bReference 24.

  • cReference 7.

  • dReference 26.