Renal cell carcinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

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Overview

The pathophysiology of renal cell carcinomas plays an important role in differentiating between the types of renal cell carcinomas and in choosing appropriate targeted medical therapies. Sporadic forms of clear cell renal carcinomas, the most common form of renal cell carcinomas, have similar pathophysiological mechanisms to those of von Hippel-Lindau (VHL) disease. On the other hand, MET proto-oncogene seems to play a role in the disease pathogenesis of papillary forms of renal cell carcinoma. Uniquely also, oncocytomas are benign tumors that arise from type A intercalated cells, whereas chromophobe renal cell carcinoma arises from type B intercalated cells.

Pathophysiology

Sporadic Forms of Clear Cell Renal Carcinoma

  • Although Von Hippel-Lindau (VHL), an autosomal dominant disorder, is characterized by the mutation of the VHL gene - a tumor suppressor gene - and the consequential development of renal clear cell carcinomas following the silencing of the remaining normal VHL gene, the gene itself has been identified to be similarly responsible of the development of sporadic forms of renal clear cell carcinomas.[1]

Familial Forms of Clear Cell Renal Carcinoma

  • The translocation and loss of chromosome 3p at the site 3p14 is hypothesized to be responsible for the development of renal clear cell carcinomas in other familial forms not related to VHL gene.[14]

Papillary Renal Cell Carcinoma

Papillary renal cell carcinoma has 2 histologic types:

Types of Papillary Renal Cell Carcinoma[1]
Type Cell Size Cytoplasm Nucleus Nucleolus
Type I Small Pale Small and oval Indistinct
Type II Large Abundant eosinophilic Large and spherical Distinct
Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma. N Engl J Med. 2005 Dec 8;353(23):2477-90

Oncocytoma and Chromophobe Renal Cell Carcinoma

Collecting Duct Renal Cell Carcinoma

References

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