Renal Cell Cancer
Renal cell carcinoma constitutes about 3% of all malignancies, so it’s not a common tumor. There’s about 25,000 cases annually in the United States that that results in about 10,000 deaths. There’s a male to female predominance of 2:1 and there’s a similar incidence in both black and white populations. It is a disease of the elderly; most of the cases present in ages 50-70, although certainly presentations in younger patients occur. The youngest patient I’ve had is somebody who is 16-years-old when he developed a renal cell carcinoma. There is an increased risk associated with cigarette smoking and the disease occurs in sporadic and familial forms.
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Now the von Hippel-Lindau syndrome is not a major health problem in the United States but it was useful in defining the genetic abnormalities that occur in both an inherited form of renal cell carcinoma as well as an acquired form. In general, those abnormalities occur on chromosome 3. This just summarizes some of the features of the von Hippel-Lindau syndrome. A multi-focal, bilateral clear cell carcinoma of the kidney occurs in about 35% of the patients with this syndrome. In your hand-out there’s a diagram of where the genetic abnormalities occur on chromosome 3 in VHL. Chromosomal abnormalities in the same area occur in patients with acquired clear cell carcinomas of the kidney as well. There is also a hereditary form of renal cell carcinoma which manifests as papillary tumors. These chromosomal abnormalities are on chromosome 8 and the median age of patients with this tumor is 52-years. Now there is a third, what I would consider hereditary, form of renal cell carcinoma and this is a much rarer form of the disease. These are renal medullary carcinomas and they occur in young patients with the sickle cell trait. Advanced presentation is typical and these tumors typically pursue a very aggressive course. The median survival is 15 weeks. As these things go, I saw three cases of this syndrome in the space of about four months when I was at the Lombardi Cancer Center and then didn’t see any others for the remaining time that I was there. So it is a fairly uncommon presentation but if you have a young patient, especially if they are African-American, it might be useful to check them for sickle cell trait; especially if they have a medullary-type of histology.
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These are the subtypes of renal cell carcinoma. The majority of the renal cell carcinomas are what we call clear cell carcinomas. About 70% of patients. The papillary version, which is also referred to as a chromophilic tumor, occurs in about 20% of the patients. Chromophobics are 5% and then other histology’s, including the renal medullary carcinomas and the transitional cell carcinomas and the collecting duct tumors, represent the other 5%. There’s a sarcomatoid variant which I’ll talk about in a little bit, which is actually a variant of the clear cell carcinomas.
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