Table 1:
RASopathy | Most Common Cancer Type(s) |
Approximate Incidence |
---|---|---|
Costello syndrome (HRAS) | General | ~40-fold increase over general population |
Bladder cancer | ~1.5% | |
Neuroblastoma | ~2% | |
Rhabdomyosarcoma | ~7% | |
Noonan syndrome (PTPN11; e.g., codon 61 or T73I), (KRAS; e.g., T58I) | JMML, rhabdomyosarcoma, ALL, neuroblastoma, brain tumors | ~8-fold increase over general population |
Noonan syndrome (SOS1, RAF1, RIT1, SOS2, RRAS, LZTR1, BRAF) | Case reports of ALL, neuroblastoma, rhabdomyosarcoma | Unclear risk |
Noonan syndrome with multiple lentigines (PTPN11, RAF1, BRAF, MAP2K1) | Case reports of AML, ALL, neuroblastoma, melanoma | Possibly mildly elevated, although unclear how much so |
Noonan syndrome with loose anagen hair (PPPC1B, SHOC2) | Case reports of neuroblastoma, myelofibrosis, t cell lymphoma | Possibly mildly elevated, although unclear how much so |
CBL syndrome (CBL) | JMML | Elevated, but unclear how much so |
Cardiofaciocutaneous syndrome (BRAF, MAP2K1, MAP2K2, KRAS) | Case reports of rhabdomyosarcoma, ALL, lymphoma | Possibly mildly elevated, although unclear how much so |
Legius syndrome (SPRED1) | Case reports of pediatric leukemias | Not believed to be elevated |