Presentation
Abdominal pain and palpable masses on physical exam.
Patient Data
Both kidneys are enlarged and contain multiple varying-sized cystic lesions. Some cysts show thick irregular enhancing walls or eccentric enhancing solid components.
In addition, a sizable heterosignal mass with central necrotic changes and heterogeneous enhancement is seen in the posterior aspect of the pancreatic head that extends into the porta hepatis and subhepatic regions.
A high T2W mass in segment VII of the liver also shows early peripheral nodular enhancement with centripetal extension suspected for hemangioma.
A little sludge is observed in the dependent part of the gallbladder.
Several small thin-walled cysts are noted in the pancreas.
Both kidneys are markedly enlarged and polycystic. Some cysts show wall thickening and irregularity with enhancing solid components. There are also multiple small calcified foci in and between the cysts.
A large well-defined lobulated mass with internal necrotic changes is noted between the pancreas head and right kidney that extends into porta hepatis. There is no sign of local invasion to adjacent structures and no vascular encasement. Additionally, several small cysts are present in the pancreas.
A low-attenuation hepatic lesion is depicted in segment VII of the liver, revealing early peripheral, nodular enhancement and centripetal filling, most consistent with hemangioma.
Fat containing midline supraumbilical hernia is seen.
Case Discussion
Features on MRI and follow-up CTscan are consistent with Von Hippel-Lindau disease, characterized by the development of numerous benign and malignant tumors in different organs, particularly in the kidney, adrenal, pancreas, liver and CNS.
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