Figure 1: Coronal images through the kidneys with the right kidney better visualized than the left. The patient is status post partial left nephrectomy for a previous renal mass. Both kidneys contain multiple simple cysts and complex cysts with hemorrhage.

Figure 2: Axial image through the abdomen at the level of the kidneys demonstrate multiple simple cysts, complex cysts with hemorrhage and solid enhancing renal masses. A solid enhancing mass is present in the upper pole of the right kidney anteriorly.

Figure 3: Axial image through the abdomen at the level of the pancreas demonstrates that the pancreas has been replaced by numerous large cystic lesions with internal septations, which likely represent serous cystadenomas.

Von Hippel-Lindau with renal cell carcinoma and pancreatic serous cystadenomas.

Von Hippel-Lindau (vHL) is an inherited autosomal dominant with incomplete penetrance and is characterized by:

• Retinal angiomatosis
• CNS hemangioblastomas (cerebellar, spinal cord, brainstem): most common cause of death
• Renal cell carcinoma: 2nd most common cause of deaths
• Cystic lesions of the kidney, liver, pancreas, and epididymis
• Papillary cystadenoma of the epididymis
• Angiomas of the liver and kidney
• Pheochromocytoma
• Endolymphatic sac tumor

In the abdomen, vHL mainly affects the pancreas, kidneys, adrenals and liver. The extent of the cystic lesions in the pancreas can vary from a few cysts to an enlarged pancreas containing numerous cysts. The pancreatic cystic lesions may represent microcystic serous cystadenomas. Pancreatic cystic lesions in vHL are often asymptomatic or associated with only mild symptoms. However, innumerable pancreatic cystic lesions may result in insulin-dependent DM. Cystic renal lesions in vHL are indistinguishable from simple cysts, and commonly affect both kidneys. There is an increased risk for developing renal cell carcinoma, as illustrated by this case, and therefore these patients need close monitoring.

References:

1. Choyke PL, Glenn GM, Walthner MM, Patronas NJ, Linehan WM, and Zabar B. Von Hippel-Lindau Disease: Genetic, Clinical and Imaging Features. Radiology. 1995, March; 194(3): 629-642.
2. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH. Von Hippel-Lindau disease. Lancet. 2003 Jun 14; 361(9374): 2059-67.