Case of the Month: November, 2012 Page 5

Answer: C. Renal cyst

Discussion Renal Von Hippel-Landau:

  • Multiple renal cysts and increased risk for renal cell carcinoma.
  • Clear cell RCC: 20-40%
    • May be multicentric or bilateral.
    • Numerous cysts may obscure cystic neoplasm or neoplasm within the wall of a cyst.
    • Screening with renal US and CT/MRI.

Follow-up:

  • The patient went to surgery and the complex renal cyst was indeed a malignancy for which partial nephrectomy was performed. There was no evidence for metastatic disease.
  • The epididymal cyst most likely represents a cystadenoma which is a characteristic lesion in VHL.
  • The pancreatic cyst is a true simple cyst in the pancreas which is rare except in the setting of VHL.

References:

  • “Principles of Internal Medicine” Harrison’s 16th edition
  • Smirniotopoulos, J Neuroimaging of phakomatoses: Sturge-Weber syndrome, tuberous sclerosis, von Hippel-Lindau syndrome Neuroimaging Clinics of North America, Volume 14, Issue 2, Pages 171-183.
  • Yousem, D et al “Neuroradiology: The Requisites” 2nd Edition 2009
  • Statdx.org
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