Table 4.

Suggested surveillance for rhabdoid tumors

Organ at riskType of mutation
GeneGermline truncatingGermline missense
SMARCB1BrainMRI q 3 months to age 5 yearsNo screening, generally no/very low risk
AbdomenConsider WBMRI to age 5 years, undetermined frequency. Ultrasound q 3 monthsNo screening, generally no/very low riska
SMARCA4BrainNo data available, risks likely very low
AbdomenNo data available, risk likely low to very low
OvaryNo data available, abdominal ultrasound q 6 months may be justified, role, if any, of MRI unknown. Preventive oophorectomy may be justified outside of the pediatric age range

Abbreviations: q, every; WBMRI, whole-body MRI.

  • aSchwannomas may result from missense mutations and may justify MRI.