Imaging diagnosis of multiple organ diseases in Von Hippel-Lindau syndrome

Objective: To investigate image feature of Von Hippel-Lindau (VHL) syndrome involved abdominal organs. Methods: Abdominal CT imaging features of 27 patients with VHL syndrome were retrospectively analyzed. 17 cases underwent abdominal unenhanced and contrast-enhanced CT. 3 cases underwent abdominal unenhanced and contrast-enhanced MRI. 7 cases underwent abdominal unenhanced and contrast-enhanced CT and MR. Results: Six of the 27 patients had family h1 story. There were 24 cases of pancreatic lesions, including 2 cases of polycystic pancreatic pancreatic neuroendocrine tumors (1 case of liver metastasis), 22 cases of polycystic pancreas. There were 14 cases of renal tumors, including 1 case of bilateral renal cell carcinoma with hamartoma, 9 cases of bilateral renal cell carcinomas, 1 case of unilateral renal cell carcinoma, 2 cases of bilateral hamartomas, and 1 case of unilateral hamartoma. 5 cases of adrenal tumors, 3 cases of bilateral adrenal pheochromocytoma, 2 cases of unilateral adrenal pheochromocytoma. There were 2 cases of common bile duct neuroendocrine tumors and 2 cases of epididymal tumor, bilateral yolk sac tumors and cystal adenoma in 1 case, respectively. The most susceptible organs in this group were the pancreas, kidneys and adrenal glands. Two or more organs were involved at the same time in 15 cases (55%). The most common type was involvement of both pancreas and kidneys (8 cases, 30%), including 6 cases of polycystic pancreas and renal carcinoma, 1 case of polycystic pancreas and hamartoma, polycystic pancreas, 1 case of renal carcinoma and hamartoma. And there were 1 case involving pancreas and common bile duct and 1 case involving pancreas and adrenal gland carcinoma. Three abdominal organs were involved in 5 case (19%). Pancreas and kidneys were involved, and adrenal gland (n = 2), epididymis (n = 2), and common bile duct (n = 1) could also be harmed. Conclusion: VHL syndrome often manifests as multiple organs involvement in the abdomen. Polycystic pancreas and renal tumors are common. Adrenal gland, common bile duct and epididymis should be focused. (authors)