History 60 yr. old Female with a history of visual changes, balance problems, nausea, and abdominal pain.60 yr. old Female with a history of visual changes, balance problems, nausea, and abdominal pain. Prior imaging studies showed hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) as well as cysts in the pancreas and a mass concerning for renal cell carcinoma in the right kidney.Prior imaging studies showed hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) as well as cysts in the pancreas and a mass concerning for renal cell carcinoma in the right kidney.

Diagnosis???

Genetic disorder involving a tumor suppressor gene on chromosome 3.Genetic disorder involving a tumor suppressor gene on chromosome 3.

Von Hippel-Lindau Disease

Pancreatic Masses The main differential diagnoses include the following: mucinous cystic neoplasm, Intraductal Papillary Mucinous Tumor, pseudocyst, focal pancreatitis, serous cystadenoma, and adenocarcinoma.The main differential diagnoses include the following: mucinous cystic neoplasm, Intraductal Papillary Mucinous Tumor, pseudocyst, focal pancreatitis, serous cystadenoma, and adenocarcinoma. HistoryHistory Radiologic FindingsRadiologic Findings

History 60 yr. old Female with a history of visual changes, balance problems, nausea, and abdominal pain.60 yr. old Female with a history of visual changes, balance problems, nausea, and abdominal pain. Prior imaging studies showed hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) as well as cysts in the pancreas and a mass concerning for renal cell carcinoma in the right kidney.Prior imaging studies showed hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) as well as cysts in the pancreas and a mass concerning for renal cell carcinoma in the right kidney. von Hippel-Lindau Diseasevon Hippel-Lindau Disease

Radiologic Findings A central stellate scar with calcification and a grapelike cluster of cysts and external lobulation strongly suggests serous cystadenoma.A central stellate scar with calcification and a grapelike cluster of cysts and external lobulation strongly suggests serous cystadenoma.

Pathology and Presentation Serous cystadenoma is a benign tumor lined by a uniform layer of glycogen-secreting cuboidal cells.Serous cystadenoma is a benign tumor lined by a uniform layer of glycogen-secreting cuboidal cells. Most patients are asymptomatic, those that do present have nonspecific symptoms such as abdominal pain, nausea, vomiting, or a palpable abdominal mass.Most patients are asymptomatic, those that do present have nonspecific symptoms such as abdominal pain, nausea, vomiting, or a palpable abdominal mass. Associated with von Hippel-Lindau disease.Associated with von Hippel-Lindau disease.

Take Home Points von Hippel-Lindau Disease: Associated with serous cystadenomas of the pancreas, renal cell carcinoma, and hemangioblastomas of the CNS and Retina.von Hippel-Lindau Disease: Associated with serous cystadenomas of the pancreas, renal cell carcinoma, and hemangioblastomas of the CNS and Retina. Classic Radiologic Presentation of Serous Cystadenoma of the Pancreas: A central stellate scar with calcification and a grapelike cluster of cysts and external lobulationClassic Radiologic Presentation of Serous Cystadenoma of the Pancreas: A central stellate scar with calcification and a grapelike cluster of cysts and external lobulation