Imaging appearances of von Hippel-Lindau disease

Objective To assess imaging appearances of von Hippel-Lindau disease (VHLD). Methods The clinical and imaging data of ten patients who met VHLD gene diagnostic criteria were retrospectively analyzed.Seven underwent CT abdominal scan,three underwent MRI abdominal scan.All patients underwent MRI cranial scan.Eight patients were treated by surgery,and three of them underwent endoscopic ultrasound guided aspiration biopsy of pancreatic tumors. Results All patients had multiple cysts of the pancreas,eight patients suffered multiple cysts of kidneys.Three patients suffered multiple islet cell tumors of pancreas: one was accompanied with pancreatic serous cystadenoma,another was accompanied with left adrenal pheochromocytoma.Three patients had bilateral renal cell carcinoma (one of them had the right kidney cut off),one patient had right renal cell carcinoma.Two patients suffered cerebellar hemangioblastomas,and one of them was accompanied with mutiple spinal hemangioblastomas and the left retinal hemangioblastoma.Two patients with multiple cysts of pancreas and kidneys had not been found any tumor. Conclusion Multiple cysts of pancreas and kidney are highly suggestive of VHLD.At the same time,endocrine tumors of pancreas,serous cystadenoma of pancreas,adrenal pheochromocytoma,renal carcinomas,multiple hemangioblastomas of central nervous system and retinal hemangioblastoma can also occur in VHLD. Key words: Pancreatic neoplasm; Hemangioblastoma; Renal neoplasms