Table 2.
Proto-Oncogenes and Tumor Suppressor Genes in Pediatric Malignancies
| Oncogene Family | Proto-oncogene | Chromosome location | Tumors |
|---|---|---|---|
| Growth Factors and Receptors | |||
| erb B2 | 17q21 | Glioblastoma | |
| trk | 9q22 | Neuroblastoma | |
| Protein Kinase | |||
| src | 7p11 | Rhabdomyosarcoma, Osteosarcoma, Ewing sarcoma | |
| Signal Transducers | |||
| H-ras | 11p15.1 | Neuroblastoma | |
| Transcription Factors | |||
| c-myc | 18q24 | Burkitt lymphoma | |
| N-myc | 2p24 | Neuroblastoma | |
| Syndrome | Tumor suppressor gene | Chromosome location | Tumors |
|---|---|---|---|
| Familial polyposis coli | APC | 5q21 | Intestinal polyposis, Colorectal cancer |
| Familial retinoblastoma | RB | 13q24 | Retinoblastoma, Osteosarcoma |
| WAGR* | WT1 | 11p13 | Wilms tumor |
| Denys-Drash† | WT1 | 11p13 | Wilms tumor |
| Beckwith-Weidemann‡ | WT2 (?) | 11p15 | Wilms tumor, Hepatoblastoma, Adrenal tumors |
| Li-Fraumeni | p53 | 17q13 | Multiple (see text) |
| Neurofibromatosis type 1 | NF1 | 17q11.2 | Sarcomas, breast cancer |
| Neurofibromatosis type 2 | NF2 | 22q12 | Neurofibroma, Neurofibrosarcoma |
| Brain tumor | |||
| Von Hippel-Lindau | VHL | 3p25-26 | Renal cell cancer, Pheochromocytoma |
| Retinal angioma, Hemangioblastoma |
*
WAGR: Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation
†
Denys-Drash: Wilms tumor, pseudohermaphroditism, mesangeal sclerosis, renal failure
‡
Beckwith-Weidemann: multiple tumors, hemihypertrophy, macroglossia, hyperinsulinism
Reprinted with permission from Davidoff AM. Principles of pediatric oncology/Genetics of cancer. In: Grosfeld JL, O'Neill JA, Fonkalsrud EW, Coran AG (Eds.) Pediatric Surgery, 6th Edition, Elsevier Science, 2006