Table 4.

Proposed vHL tumor surveillance regimen with an emphasis on the pediatric age range

TumorRecommended surveillanceAge to beginInterval
Retinal HBEye exam including retinaaBirthAnnual
PHEOBlood pressure at all medical visitsb2 years
PFMc,d,e,f or 24-h urine fractionated metanephrinesg2 yearsAnnual
ELSTAudiogram5 yearsBiennial
CNS HBMRI brain with and without contrasth8 yearsBienniali
MRI spine with contrast
RCCMRI abdomenj10 yearsAnnual
Pancreatic NETMRI abdomen10 yearsAnnual
  • Abbreviations: HB, hemangioblastoma; PFM, plasma-free metanephrines.

  • aDuring childhood, ophthalmologic examination should be performed by an ophthalmologist with experience in pediatric retinal examination.

  • bBlood pressure in children should be assessed using age- and height-specific normative ranges (https://www.nhlbi.nih.gov/files/docs/guidelines/child_tbl.pdf).

  • cReference to pediatric reference intervals for plasma (71, 72) and urine (73) metanephrines should be considered.

  • dIdeally, to limit false positive results, PFMs should be collected from an indwelling venous catheter after patient has been lying supine for ≥30 minutes. Clinicians may elect to bypass this approach, but marginally elevated results should prompt repetition of testing under ideal conditions.

  • eSeveral foods and medications may interfere with metanephrine analysis and should be avoided prior to testing. These are summarized in Supplementary Table S1.

  • fRecommended action based on plasma metanephrines:

    • –Confirm interfering agents were avoided prior to testing (Supplementary Table S1).

    • –If ≥4× upper limit of reference range: consistent with disease, proceed with imaging to localize lesion.

    • –If 2×–4× upper limit of reference range: repeat testing in 2 months.

    • –If marginally elevated: repeat testing in 6 months or consider clonidine suppression test to exclude false positivity (74, 75).

  • gTwenty-four–hour urine fractionated metanephrines are an acceptable alternative to plasma metanephrines once patients are continent of urine.

  • hIncluding thin cuts through internal auditory canals.

  • iSome providers may choose to advance the frequency of CNS imaging to annually starting in adolescence due to retrospective data describing relatively quick growing hemangioblastomas in between every-2-year scans.

  • jMRI surveillance for RCC and pancreatic NET may be part of the same study, provided that dedicated renal sequences are included.