Here we reviewed the molecular genetic mechanism in the development of 4 types of human hereditary kidney cancers. Loss of function of the VHL disease gene is responsible for the von Hippel-Lindau disease and major portion of sporadic clear cell renal carcinoma. Activated c-Met oncogene is responsible for the development in some cases of hereditary papillary renal cell carcinomas and sporadic papillary renal carcinomas. There are several cases of familial renal carcinoma in that translocations of chromosome 3 p are demonstrated. The molecular genetic mechanism of this disease is not known.
Several reports show the development of renal cell carcinoma in Tuberous sclerosis patients.
TSC 1 or TSC 2 gene may be responsible for these tumors. The detail in this disease not well known. Molecular genetic analyses for hereditary renal cancer identified several oncogenes and tumor suppressor genes in hereditary as well as sporadic renal carcinomas. Future studies may reveal new category of oncogenes or tumor suppressor genes that are involved in the human kidney cancer development. In contrast, the phenotypes of A498 VHL-negative RCC cells were weaker, and isogenic RCC cells ectopically expressing wt VHL did not respond at all.