Renal cell carcinomas in von Hippel-Lindau disease; tumor detection and management

Twenty-seven renal cell carcinomas (RCCs) found in one family affected with von Hippel-Lindau disease were examined using ultrasound (US), CT, MRI and angiography. The sensitivity of the tumor detection using different imaging modalities was evaluated by macroscopic pathology (solid or cystic) and size (exceeding 2cm in diameter or not). In 18 of the RCC's exceeding 2cm in diameter (eight solid and ten cystic), all lesions were detected on US, CT, and MRI. However, on angiography, solid RCCs were detected in 88%, and cystic RCCs were detected in 60%. In nine RCCs less than 2cm in diameter (seven solid and two cystic), solid RCCs were detected in 86% on US, 86% on CT, 80% on MRI, and 43% on angiography, but cystic RCCs were detected in 50% on only CT and MRI. From the pathologic correlation, even renal simple cystic lesions in VHL are considered premalignant lesions and they had better be removed if the residual renal function after surgery is preserved. In case of the observation, they should be followed carefully using thin slice thickness dynamic CT to discover the wall irregularity, septation and irregular contour.