von Hippel-Lindau disease (vHL)

Case contributed by Dr Maxime St-Amant

Presentation

Abdominal pain.

Patient Data

Age: 25
Gender: Female

CT scan shows multiple non-enhancing pancreatic cysts and bilateral renal angiomyolipomas, highly suggestive features of von Hippel-Lindau disease. There is also a small enhancing exophytic lesion involving the upper pole of the right kidney; a renal cell carcinoma must be excluded.

Case Discussion

Patient with VHL are at increased risk for renal cell carcinoma, particularly clear cell RCCs. The diagnosis was later confirmed (clear cell renal cell carcinoma) and the right upper pole renal lesion was surgically removed.

Pancreatic cysts are rare isolated findings. There is a wide differential diagnosis discussed here.

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Case information

rID: 18932
Case created: 28th Jul 2012
Last edited: 28th Feb 2016
Inclusion in quiz mode: Included

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