Von Hipple-Lindau disease

Presentation

History of surgical resection of a small right renal cell carcinoma.

Patient Data

Age: 35 years
Gender: Female
CT

Multiple variable-sized pancreatic and bilateral renal cysts are noted. A small subcapsular hepatic focal lesion is also seen at segment VII possibly small cyst.

MRI

A well-defined intra-axial lesion is seen centred on the posterior aspect of the medulla oblongata appears of low signal intensity on T1, bright signal intensity on T2 with posterior enhancing intra-mural small linear area.

Case Discussion

Von Hipple-Lindau disease is a rare type of phakomatosis with various CNS, renal, pancreatic and head and neck abnormalities like hemangioblastoma, renal cysts, renal cell carcinoma, pheochromocytoma and pancreatic cysts as well as cystadenoma and cystadenocarcinoma.

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Case information

rID: 53675
Case created: 30th May 2017
Last edited: 1st Jun 2017
Inclusion in quiz mode: Included

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