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