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Von Hippel-Lindau syndrome - renal manifestations

Case contributed by Chris O'Donnell
Diagnosis certain

Presentation

Known VHL. Screening for renal masses. Neurogenic bladder. MRI performed in order to reduce radiation exposure.

Patient Data

Age: 30 years
Gender: Male

Multiple simple renal cysts as well as multiple mixed solid and cystic renal neoplasms. 

The pancreas also contains a huge number of cysts throughout.

No pheochromocytoma.

Case Discussion

This case has a full hand of the VHL syndrome, i.e. multiple simple renal cysts as well as multiple mixed solid and cystic renal neoplasms.  The pancreas also contains a huge number of cysts throughout.

The patient has had a spinal hemangioblastoma removed causing the neurogenic bladder (note thoracic laminectomy and changes in the spinal cord on the T2 axial series).

No pheochromocytoma.

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