Pathophysiology
Renal cell carcinoma originates in the proximal renal tubular epithelium. Kidney cancer occurs in a hereditary and nonhereditary (most frequently the latter), and both forms are associated with structural alterations of the short arm of chromosome 3 (3p).
Is associated with genes, tumor suppressor genes (VHL, TSC) or oncogenes (MET).
There are at least 4 hereditary syndromes associated with renal cell carcinoma:
1) The von Hippel-Lindau (VHL), which is a familial syndrome associated with various cancers, among which we quote:
* Renal cell carcinoma with clear cell histology
* Pheochromocytoma
* Pancreatic cysts and tumors of islet cell
* Retinal angiomas
* The central nervous system hemangioblastomas
* Endolymphatic sac tumors
* Epididymal cystadenomas
Renal cell carcinoma develops in nearly 40% of patients with von Hippel-Lindau and is a major cause of death among these patients.
2) hereditary papillary renal carcinoma (HPRC) is an inherited disorder with an autosomal dominant pattern, affected individuals develop a severe form of bilateral papillary renal carcinoma and multifocal (ie develops MABOS kidney disease and in different parts of each kidney at a time).
3) familial renal oncocytoma (FRO) associated with Birt-Hogg Dube (BHDS), this syndrome also develops multifocal or bilateral forms. Patients with this syndrome have an inherited predisposition to develop benign tumors of hair follicle (ie fibrofolliculomas), predominantly in the face, neck and upper trunk, and are at risk of developing renal tumors, colon tumors or polyps and lung cysts.
4) hereditary renal carcinoma (HRC).
Incidence of renal cancer
We have no statistical Peru. In the United States of America estimated 60 000 new cases with 13 deaths during 2009.