Table 1.

Surveillance recommendations for rhabdoid tumor predisposition syndrome (RTPS) carriers.

Foulkes et al. (37)

Teplick et al. (55)

Germline truncating mutations: SMARCB1  - Brain: MRI every 3 months to age 5 years  - Abdomen: Ultrasound every 3 months through 5 years. Consider WB-MRI, undetermined frequency SMARCA4  - Brain: No data available, risks likely very low  - Abdomen: No data available, risk likely low to very low  - Ovary: No data available, abdominal ultrasound every 6 months may be justified, role, if any, of MRI unknown. Preventive oophorectomy may be justified outside of the pediatric age range Germline missense mutations: No screening, generally no/very low risk

- From 0–1 year: is recommended abdominal US every 2 to 3 months and head US monthly - From 1–4 years: abdominal US every 6 months. Brain and spine MRI every 6 months

MRI, magnetic resonance imaging; WB-MRI, whole body magnetic resonance imaging; US, ultrasound.