Comprises 90% of kideny cancers in adults.

Most common in the sixth to seventh decades of life.

More common in males (3:1).

Occurs in association with the von Hippel-Lindau syndrome.

Clinically, the tumor may present with hematuria (90%), fever, or paraneoplastic syndrome. Cushing's syndrome, feminization, masculinization, polycythemia, eosinophilia, leukemoid reaction, hypercalcemia, hypertension, and amyloidosis are amongst the conditions seen as a paraneoplastic syndrome in patients with renal cell carcinoma.

Grossly, the tumor appears as a round, 3-15 cm, mass with a gray-white to yellow cut surface along with areas of hemorrhage and necrosis. The tumor often invades the renal vein and may extend into the inferior vena cava to the right side of the heart.

Microscopically, the tumor is composed of cells containing glycogen and lipid that in view of loss of these substances during processing appear as clear cells to cells that contain numerous mitochondria and therefore appear as granular cells at the light microscopic level. The cytologic atypia varies from mild to severe and areas of hemorrhage and necrosis is common in these tumors.