CANCER PREDISPOSITION

Question 1

Automatic diagnoses that should trigger germline TP53 Testing

Answer 1

1. Anaplastic RMS (75%)
2. SHH Medulooblastoma (75%)
3. Adrenocorticocarcinoma <15 years (50%)
4. Choroid Plexus Carcinoma (50%)
5. Hypodiploid ALL (50%)
6. Early onset breast cancer (10%)

Question 2

Li Fraumeni

- clinical diagnostic criteria

Answer 2

Proband dx with sarcoma before age 45

First degree relative with cancer before age 45

First or second degree relative with cancer before age 45 or a sarcoma at any age.

See Chompret guidelines