HistoryHistory

• 60 yr. old Female with a history of 60 yr. old Female with a history of visual changes, balance problems, visual changes, balance problems, nausea, and abdominal pain.nausea, and abdominal pain.

• Prior imaging studies showed Prior imaging studies showed hemangioblastomas in the cerebellum, hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) and kidneys (none in the retina – yet) as well as cysts in the pancreas and a as well as cysts in the pancreas and a mass concerning for renal cell mass concerning for renal cell carcinoma in the right kidney.carcinoma in the right kidney.

Diagnosis???Diagnosis???

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

Von Hippel-Lindau Von Hippel-Lindau DiseaseDisease

Pancreatic MassesPancreatic Masses

• The main differential diagnoses include The main differential diagnoses include the following: mucinous cystic the following: mucinous cystic neoplasm, Intraductal Papillary neoplasm, Intraductal Papillary Mucinous Tumor, pseudocyst, focal Mucinous Tumor, pseudocyst, focal pancreatitis, serous cystadenoma, and pancreatitis, serous cystadenoma, and adenocarcinoma.adenocarcinoma.

• HistoryHistory• Radiologic FindingsRadiologic Findings

HistoryHistory

• 60 yr. old Female with a history of visual 60 yr. old Female with a history of visual changes, balance problems, nausea, and changes, balance problems, nausea, and abdominal pain.abdominal pain.

• Prior imaging studies showed Prior imaging studies showed hemangioblastomas in the cerebellum, and hemangioblastomas in the cerebellum, and kidneys (none in the retina – yet) as well as kidneys (none in the retina – yet) as well as cysts in the pancreas and a mass concerning cysts in the pancreas and a mass concerning for renal cell carcinoma in the right kidney.for renal cell carcinoma in the right kidney.

• von Hippel-Lindau Diseasevon Hippel-Lindau Disease

Radiologic FindingsRadiologic Findings

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

Pathology and Pathology and PresentationPresentation

• Serous cystadenoma is a benign tumor Serous cystadenoma is a benign tumor lined by a uniform layer of glycogen-lined by a uniform layer of glycogen-secreting cuboidal cells.secreting cuboidal cells.

• Most patients are asymptomatic, those Most patients are asymptomatic, those that do present have nonspecific that do present have nonspecific symptoms such as abdominal pain, symptoms such as abdominal pain, nausea, vomiting, or a palpable nausea, vomiting, or a palpable abdominal mass.abdominal mass.

• Associated with von Hippel-Lindau Associated with von Hippel-Lindau disease.disease.

Take Home PointsTake Home Points

• von Hippel-Lindau Disease: Associated von Hippel-Lindau Disease: Associated with serous cystadenomas of the pancreas, with serous cystadenomas of the pancreas, renal cell carcinoma, and renal cell carcinoma, and hemangioblastomas of the CNS and hemangioblastomas of the CNS and Retina.Retina.

• Classic Radiologic Presentation of Serous Classic Radiologic Presentation of Serous Cystadenoma of the Pancreas: A central Cystadenoma of the Pancreas: A central stellate scar with calcification and a stellate scar with calcification and a grapelike cluster of cysts and external grapelike cluster of cysts and external lobulation lobulation