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Radiology Resident Case of the Week

Von-Hippel Lindau disease

September 19, 1996

Akira Fujikawa
Peer Review Status: Not reviewed
History

Clinical Sx:
s/p cerebeller tumor resection

Etiology/Pathophysiology

Pathology:

Miscellaneous

Imaging
Multiple pancreatic cysts and a few of renal cysts on abdominal CT. well enhanced posterior fossa tumor and foci in the cervical/thracic spinal cord on MRI.

DDX
The differential of multiple pancreatic cysts include inflammatory cysts(pseudocysts-mosy common, abscess, echinococcus cyst), Neoplastic cysts(microcystic adenoma, cystic teratoma, mucinous cystic ca., solid and papillary epitherial neoplasm, lymphoma, etc.), and congenital cysts( adult polycystic kidney disease, von Hippel-Lindsu disease, cystic fibrosis). In this case, cerebeller and spinal hemangioblastoma suggest von Hippel-Lindau disease. CNS hemangioblastoma and at least one lesion of VHL complex satisfies diagnostic criteria.

Key references
Cystic mass of the pancreas Radiographics 1992;12:673-686

ACR Code

Keywords:
pancreatic cyst

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