Renal cell carcinoma associated with Von Hippel-Lindau disease

a) the most common malignant tumor in Von Hippel-Lindau disease. Usually a clear cell carcinoma

b) lifetime risk for renal cell carcinoma (RCC) in Von Hippel-Lindau disease:≈70%.

c) the growth rate of RCC is high variable

d) RCC is the cause of death in 15–50% of patients

e) metastases respond poorly to chemotherapy and radiation

f) bilateral and multiple lesions are common

g) partial nephrectomy or tumor enucleation is preferred to avoid/delay dialysis and transplantation

h) nephron-or renal-sparing surgery recommended for tumors less < 3cm

i) promising techniques: cryo- and radiofrequency ablation of tumors < 3 cm