Description

• Renal cell carcinoma (RCC), (aka hypernephroma or Grawitz tumor), accounts for 3–4% of all adult cancers and 2.3% of all cancer deaths; 7th most common malignant tumor in men and 9th in women
• Characterized by obscure and varied presentations, including paraneoplastic syndromes, vascular findings, and uncommon metastatic sites
• Early, aggressive surgical management provides the best opportunity for cure.
• System(s) affected: Renal/Urologic

Epidemiology

• Predominant age: Patients in 5th–7th decades; median age at diagnosis is 66 years.
• Predominant sex: Male > Female (1.6:1)
• Age-adjusted incidence increasing 3%/yr, likely due in part to increased detection as an incidental finding on imaging studies
• In males, 10.7 new cases of RCC per 100,000 population per year vs. 6.5 in females
• Between 1975 and 1998, incidence among African Americans increased by 4.5% compared with 2.9% among Caucasians; reasons are unclear.

Risk Factors

• Smoking, active and passive (increases relative risk by 2–3)
• Obesity (linear relationship in women)
• Hypertension (antihypertensive medications are not independently associated with RCC)
• End-stage renal failure
• Acquired renal cystic disease
• Tuberous sclerosis
• HIV infection
• Urban environment
• Heavy metal exposure (cadmium, lead)
• Environmental toxin exposure (asbestos, petroleum by-products, chlorinated solvents)

• 2–3% of cases are familial, with several autosomal-dominant syndromes described.
• Oncogenes localized to the short arm of chromosome 3 may have etiologic implications. Chromosome 3p12–p26 is specific for clear cell RCC.
• People with HLA types Bw44 and DR8 are prone to develop RCC. These are rare familial RCCs.
• Hereditary papillary RCC is an autosomal-dominant form of disease associated with multifocal papillary renal tumors and a 5:1 male predominance.

General Prevention

Smoking may contribute to 1/3 of all cases.

Etiology

Unknown

Commonly Associated Conditions

• Von Hippel–Lindau disease: 30–45% of these patients develop clear cell tumors.
• Tuberous sclerosis: Associated primarily with angiomyolipoma and clear cell tumors
• Sickle-cell trait: With few exceptions, renal medullary tumor is seen in young African American males with sickle-cell trait.
• Adult polycystic kidney disease
• Horseshoe kidney
• Acquired renal cystic disease from chronic renal failure