MR imaging of Hippel-Lindau disease: value of gadopentetate dimeglumine.

Hippel-Lindau disease is an autosomal dominant disorder characterized by tumors of the central nervous system and abdominal viscera. Frequent multisystem radiologic evaluation of persons at risk is desirable. Twenty-seven patients with Hippel-Lindau disease or a family history of the disease were examined with both unenhanced and gadopentetate dimeglumine-enhanced magnetic resonance (MR) imaging to study the usefulness of the contrast medium in the evaluation of these patients. The MR studies were correlated with computed tomographic (CT) scans in seven patients and cerebral angiograms in five. Twelve patients had hemangioblastomas in the brain, and eight of these patients also had spinal cord lesions (most were multiple), well demonstrated with gadopentetate dimeglumine. Contrast-enhanced MR imaging enabled detection of more central nervous system lesions and provided better delineation than unenhanced MR imaging, CT, or angiography. In addition, four patients with multiple renal cysts seen on CT scans and unenhanced MR images had enhancing lesions that were later proved to be renal cell carcinoma at angiography and/or surgery. Four patients had cystic lesions in the pancreas that did not enhance and were later proved to be pancreatic cysts. The authors conclude that gadopentetate dimeglumine-enhanced MR imaging appears to be a useful method for evaluating and following up patients with Hippel-Lindau disease.