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

Lists

Cystic Pancreatic Lesions

• Acinar cell cystadenocarcinoma
• Acinar cell cystadenoma
• Enterogenous duplication cyst
• Epidermoid cyst in intrapancreatic accessory spleen
• Intraductal oncocytic papillary neoplasm
• Intraductal papillary mucinous neoplasm
• Islet cell / well differentiated neuroendocrine tumor (occasionally cystic)
• Lymphangioma
• Lymphoepithelial cyst
• Mucinous cystic neoplasm
• Paraduodenal wall cyst
• Pseudocyst
• Serous cystadenocarcinoma
• Serous oligocystic adenoma
• Serous microcystic adenoma
• Solid and cystic hamartoma
• Solid pseudopapillary neoplasm
• Squamoid cyst of pancreatic ducts
• von Hippel Lindau associated cystic lesions

Bibliography

• Neumann HP, Dinkel E, Brambs H, Wimmer B, Friedburg H, Volk B, Sigmund G,
  Riegler P, Haag K, Schollmeyer P, et al. Pancreatic lesions in the von Hippel-Lindau syndrome. Gastroenterology. 1991 Aug;101(2):465-71.
• Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications. Arch Pathol Lab Med. 2009 Mar;133(3):423-38.
• Périgny M, Hammel P, Corcos O, Larochelle O, Giraud S, Richard S, Sauvanet A, Belghiti J, Ruszniewski P, Bedossa P, Couvelard A. Pancreatic endocrine microadenomatosis in patients with von Hippel-Lindau disease: characterization by VHL/HIF pathway proteins expression. Am J Surg Pathol. 2009 May;33(5):739-48.
• Corcos O, Couvelard A, Giraud S, Vullierme MP, Dermot O'Toole, Rebours V, Stievenart JL, Penfornis A, Niccoli-Sire P, Baudin E, Sauvanet A, Levy P, Ruszniewski P, Richard S, Hammel P. Endocrine pancreatic tumors in von Hippel-Lindau disease: clinical, histological, and genetic features. Pancreas. 2008 Jul;37(1):85-93.