Pancreatic and renal involvement in von Hippel-Lindau disease: Imaging findings

Objective: To analyze the imaging features and to enhance the understanding of pancreatic and renal involvement in von Hippel-Lindau disease (VHLD). Methods: CT and MRI appearances and clinical data of six patients with pancreatic and renal involvement in VHLD were studied retrospectively. Six patients underwent CT scanning, and two of them also had MRI. Results: Pancreatic cysts found in all six patients varied from several small cysts to cystic replacement of the entire gland. Calcifications were detected in four patients. Multiple bilateral renal lesions were detected in six patients. The renal lesions were classified as cystic, cystic with solid components and solid. Multiple combined renal lesions were found in five patients, and multiple simple cysts in one patient. Unilateral nephrectomy was performed in two patients, and the renal masses were diagnosed as clear cell carcinoma by pathology. Bile carcinoid was found in one patient, and retroperitoneal metastasis in another. Conclusion: Multiple pancreatic cysts and/or multiple and bilateral combined renal lesions are highly suggestive of VHLD.