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Von Hippel-Lindau disease (vHL)

Case contributed by Maxime St-Amant
Diagnosis certain

Presentation

Abdominal pain.

Patient Data

Age: 25 years
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

Patients 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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