History

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History

• 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.

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

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Von Hippel-Lindau Disease
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Pancreatic Masses

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

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History

• 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.
• von Hippel-Lindau Disease

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Radiologic Findings

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

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Pathology and Presentation

• 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.
• Associated with von Hippel-Lindau disease.

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Take Home Points

• 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 lobulation