Pancreatic Involvement in von Hippel Lindau Disease: A Single‐Center Experience

IntroductionVon Hippel Lindau (VHL) disease is an autosomal dominant genetic disorder, which is characterized by the development of benign and malignant tumors and cysts in multiple organs.Most commonly affected organs are cerebellum, eyes, spinal cord, kidneys, adrenal glands, and pancreas [1].Pancreatic manifestations range from simple cysts and serous cystadenomas to potentially malignant pancreatic neuroendocrine tumors (PNETs) [2,3].This study describes the clinical features and pancreatic manifestations of VHL patients with pancreatic involvement managed at our center. | MethodsThis is a retrospective observational study conducted at the Department of Gastroenterology and Hepatology, Istanbul University, Istanbul Medical Faculty.All patients with genetically or clinically confirmed VHL disease were screened.15 patients with confirmed pancreatic involvement were included in the study.Written informed consent was obtained from each patient.Data regarding demographic characteristics, family history, age at VHL diagnosis, and pancreatic and extra-pancreatic organ involvement were extracted from electronic health records.This study was descriptive in nature.Categorical variables were summarized using counts and percentages, while continuous variables were reported as means with ranges.No inferential statistical analyses were performed due to the small sample size.