Updated 3 July 2007
Rarely, renal cell carcinoma (RCC) occurs in a familial form as described above and up to 45% of patients with von Hippel-Lindau syndrome
will develop renal cell carcinoma. The familial syndromes are associated with renal cell carcinoma in younger age groups and disease may be bilateral. The occurrence of RCC at young age should prompt a thorough family history and a referral to an expert center. Annual ultrasound screening for patients with hereditary syndromes is recommended.
Although renal cell carcinoma is more common in smokers and patients with autosomal dominant polycystic kidney disease, screening has not been shown to be effective for these risk groups.