| Summary: | Renal cell carcinoma represents a heterogeneous group of tumors, the most common of which is clear cell adenocarcinoma. The annual incidence of this tumor appears to be rising and approximately 12,000 individuals die from this cancer annually in the United States. One third of patients who present have metastatic disease at the time of diagnosis, and another 40% who undergo nephrectomy will ultimately develop this complication. Over the past 10 years, a significant amount of new information concerning the epidemiology, mole- lar and immunologic characteristics, and therapy for patients with these tumors has appeared. The recognition that inherited forms of renal cancer exist, and that chromosomal abn- malities can be identified in these tumors, suggested a genetic basis for renal cell carcinoma. The familial cancer syndrome, Von Hippel Lindau disease, provided the setting in which the genetic abnormalites associated with the development of renal cancer were first described. Abnormalities of the VHL gene have also been detected in sporadic clear cell carcinoma, and it has now been recognized that approximately 80 % of these tumors will demonstrate ch- acteristic alterations. Currently the functions of the VHL protein are being investigated, and the biology of clear cell carcinoma of the kidney is under study. Additionally, papillary carcinomas of the kidney appear to express different molecular defects, and these are now being unraveled
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