SS  Vol.4 No.2 , February 2013
Pancreatic Involvement in Von Hippel-Lindau Syndrome: Report of a Total Pancreatectomy Case and Literature Review
Abstract: Von Hippel-Lindau (VHL) syndrome is a rare, autosomal dominantly inherited multicancer syndrome. Disease is predisposed by hemangioblastoma, renal cell carcinoma, pheochromocytoma, pancreatic islet cell and cystic tumors or epididymis tumors under the mutation or deletion at the tumor suppressor gene. Here we report a 42-year-old woman who had been operated for cerebellar hemangioblastoma and retinal angioma, and presented with recurrent, mild abdominal pain. In radiological imaging studies, multiple pancreatic cysts without nodulation were determined. Because of the mild abdominal pain and uncertain malignant potential of the cysts, total pancreatectomy was performed. She was discharged at the fifteenth day after the surgery without any problem. The patient has been followed for 62 months without any problem, and her diabetes mellitus is being controlled by medication.
Cite this paper: K. Serin, F. Yanar, O. Agcaoglu, M. Keskin, O. Ceyhan and O. Bilge, "Pancreatic Involvement in Von Hippel-Lindau Syndrome: Report of a Total Pancreatectomy Case and Literature Review," Surgical Science, Vol. 4 No. 2, 2013, pp. 151-153. doi: 10.4236/ss.2013.42029.

[1]   B. M. Kunzli, S. V. Shrikhande, M. W. Buchler, et al., “Pancreatic Lesions in Von Hippel-Lindau Syndrome: Report of a Case,” Surgery Today, Vol. 34, No. 7, 2004, pp. 626-629. doi:10.1007/s00595-004-2769-6

[2]   C. A. Friedrich, “Von Hippel-Lindau Syndrome: A Pleamorphic Condition,” Cancer, Vol. 86, No. S11, 1999, pp. 2478-2482. doi:10.1002/(SICI)1097-0142(19991201)86:11+<2478::AID-CNCR4>3.0.CO; 2-5

[3]   B. Taouli, M. Ghouadni, J. M. Correas, et al., “Spectrum of Abdominal ?maging Findings in Von Hippel-Lindau Disease,” American Journal of Roentgenology, Vol. 181, No. 4, 2003, pp. 1049-1054.

[4]   H. P. H. Neumann, E. Dinkel, H. J. Brambs, et al., “Pancreatic Lesions in the Von Hippel-Lindau Syndrome,” Gastroenterology, Vol. 101, No. 2, 1991, pp. 465-471.

[5]   E. Levine, D. L. Collins, W. A. Horton, et al., “CT Screening of the Abdomen in von Hippel-Lindau Disease: An Unusual Cause of Pancreatic and Common Bile Duct Obstruction,” American Journal of Radiology, Vol. 139, 1982, pp. 505-510.

[6]   P. R. Hammel, V. Vilgrain, B. Terris, et al., “Pancreatic ?nvolvement in vVon Hippel-Lindau Disease,” Gastroenterology, Vol. 119, No. 4, 2000, pp. 1087-1095. doi:10.1053/gast.2000.18143

[7]   T. Y. Cheng, C. H. Su, Y. M. Shyr, et al., “Management of Pacreaticlesions in Von Hippel-Lindau Disease,” World Journal of Surgery, Vol. 21, No. 3, 1997, pp. 307-312. doi:10.1007/s002689900234

[8]   B. Mukhopadhyay, A. Sahdev, J. P. Monson, et al., “Pancreatic Lesions in Von Hippel-Lindau Disease,” Clinical Endocrinology, Vol. 57, No. 5, 2002, pp. 603-608. doi:10.1046/j.1365-2265.2002.01637.x

[9]   C. M. Pyke, J. A. Heerden, T. V. Colby, et al., “The Spectrum of Serous Cystadenoma of the Pancreas Clinical, Pathologic and Surgical Aspects,” Annals of Surgery, Vol. 215, No. 2, 1992, pp. 132-139. doi:10.1097/00000658-199202000-00007

[10]   N. Agarwal, S. Kumar, J. Dass, et al., “Diffuse Pancreatic Serous Cystadenoma Associated with Neuroendocrine Carcinoma: A Case Report and Review of Literature,” Journal of Pancreas, Vol. 10, No. 1, 2009, pp. 55-58.