• ~60y, males 3:1, 98% sporadic. Familial rare.
• tubular epithelial tumour
• Both involve loss of VHL tumour suppressor gene (Von Hippel-Lindau) – Chromosome 3p. Leads to Familial Carcinoma Sy. – early age, bilateral.
• Sporadic: single tumour more common. Risk factors: Tobacco* Obesity*.
• Hereditary: multiple tumours
• Clear cells, well demarcated, grows in to renal vein

Clinical Features

• Triad: Hematuria, flank pain & Mass* (<10%)
• Hematuria (50%) most common
• Hematogenous spread
• Paraneoplastic syndromes common (Hormone)
  • PTH → Hypercalcemia
  • Erythropoietin → Polycythemia
  • Hypertension, Amyloidosis, Leucocytosis & Eosinophilia.
• 5 year survival ~40%

Gross

• large
• yellow
• hemorrhagic