[临床医学]13泌尿系统.ppt

Three most common forms Clear Cell Carcinomas Papillary Renal Cell Carcinomas Chromophobe Renal Carcinomas Three most common forms Clear cell carcinomas are the most common; associated with homozygous loss of the VHL tumor suppressor protein; tumors frequently invade the renal vein. Three most common forms Papillary renal cell carcinomas are frequently associated with increased expression and activating mutations of the MET oncogene; tend to be bilateral and multiple, and show varyingpapilla formation. Three most common forms Chromophobe renal cell carcinomas are less common; tumor cells are not as clear as in the other renal cell carcinomas. Clinically, patients may show hematuria, fever, constitutional symptoms, and /or a paraneoplastic syndrome. Renal cell carcinoma has a tendency to metastasize widely before giving rise to any local symptoms. Nephroblastoma(Wilms’ Tumor) The most common primary renal tumor of childhood, usually diagnosed between the age of 2 and 5 years. The risk of Wilms’ tumor is increased in association with at least three recognizable groups of congenital malformations exhibiting aberrations in at least two distinct chromosomal loci (Wilms’ tumor-associated genes, WT-1 and WT-2) Grossly, Wilms’ tumor tends to present as a large, solitary, well-circumscribed mass, although 10% are either bilateral or multicentric. On cut section, the tumor is soft, homogeneous, and tan to gray with occasional foci of hemorrhage, cyst formation, and necrosis. Microscopically, Wilms’ tumors are characterized by recognizable attempts to recapitulate different stages of nephrogenesis. The classic triphasic combination of blastemic, stromal, and eiphelial cell types is observed in the vast majority of lesions, although the percentage of each component is variable. Most patients present with a large abdominal mass that may be unilateral or, when very large, may extend across the midline and down into the pelvis. Hematuria, pain in the a