Table 1.
Selected inherited cancer predisposition syndromes
| Syndrome | Gene | Frequency | Primary tumor | Subsequent or related tumors | Gene-radiation interaction |
|---|---|---|---|---|---|
| Hereditary Retinoblastoma | RB1 | 1/20,000 births | Retinoblastoma | Bone sarcoma, STSa, melanoma, brain | Definite for bone and soft tissue sarcomas in the head |
| Li-Fraumeni | p53 | Rare | soft tissue sarcoma, breast cancer | Brain, leukemia, adrenocortical | Possible |
| Neurofibromatosis Type 1 | NF1 | 1/3500 births | Neurofibroma, optic pathway glioma | Gliomas, MNPSTb, STS, leukemia | Probable |
| Nevoid Basal Cell Carcinoma (Gorlin syndrome) | PTCH | Rare | Basal cell cancer | Medulloblastoma | Definite |
a
STS, soft tissue sarcoma
b
MPNST, malignant peripheral-nerve sheath tumors