Pancreatic Lesions of von Hippel Lindau Syndrome
Definition
- Serous cystic lesions and endocrine lesions may be seen in the pancreas of patients with von Hippel Lindau syndrome
Diagnostic Criteria
- A range of serous cystic lesions may be seen in 35-80% of patients
- Single cysts
- Multiple cysts throughout the pancreas (most common)
- Microscopic and macroscopic serous microcystic adenomas
- Well differentiated neuroendocrine (islet cell) tumors seen in 10-17%
- Over half have local invasion or metastasis
- Over half have clear cell features
- May be multiple
- Endocrine microadenomas reported in 72% of pancreata resected for a macroscopic endocrine tumor (Perigny)
- 5 mm or less in diameter
- Frequently multiple
- Not clear if these are also present in pancreata without macroscopic tumors
- No mucinous lesions reported
Clinical
- Treatment may not be required for cystic lesions
- No progression reported
- Endocrine neoplasms may metastasize to local lymph nodes
- Not clear if distant metastases occur
- Cystic transformation of the pancreas has also been reported in a variety of other congenital syndromes
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting : March 23, 2007
Updates: January 12, 2008; April 26, 2009; May 25, 2009; June 20, 2009
