Pancreatic Involvement in Von Hippel-Lindau Syndrome: Report of a Total Pancreatectomy Case and Literature Review

Kursat Rahmi Serin, Fatih Yanar, Orhan Agcaoglu, Metin Keskin, Ozgur Ceyhan, Orhan Bilge

Department of General Surgery, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Department of Radiology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

DOI: 10.4236/ss.2013.42029                

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.

Keywords

Von Hippel-Lindau Syndrome; Pancreatic Cyst; Malignancy

Conflicts of Interest

The authors declare no conflicts of interest.

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