This patient had renal dysfunction. Polycystic VHL simulating autosomal dominant polycystic kidney disease. Note extensive bilateral renal and pancreatic cysts. This pattern can mimic autosomal dominant polycystic kidney disease,including renal failure, however, although these cysts appear benign they commonly contain foci of malignancy within their wall. Some of these undoubtedly develop into macroscopic tumors.
Metastatic disease is the unfortunate consequence of a neglected renal tumor.
This observation has justified a conservative approach to solid masses in the kidney. More recent evidence suggests that metastatic disease can occur in lesions greater than 3 cm and the risk increases with increasing mass size. Nonetheless, ultrasound is critical to help determine whether a lesion is principally cystic or solid. Because of the extraordinary frequency of microscopic RCC in VHL we never diagnose a renal lesion as a pure cyst and consider all renal lesions suspicious for harboring cancer regardless of their imaging appearance. Contrast enhanced MRI is sometimes useful in determining whether a lesion is principally solid or cystic.