Case report: total pancreatectomy in a women with Von Hippel Lindau syndrome

Abstract

Background: The association between pancreatic disease and Von Hippel Lindau (VHL) syndrome has rarely been exposed, the main lesions in pancreatic parenchyma are serios cyst, they are usually asymptomatic lesions but when they increase in size and generate obstruction in neighboring organs is necessary to perform a surgical approach. Methods: Descriptive study, type case control, clinical case report of a patient with Von Hippel Lindau syndrome and the association with serous cysts and the requirement of total pancreatectomy. Results: Female patient in puerperium with obstructive biliary syndrome, in imaging studies is evident polycyst serous that makes messes art to perform total pancreatectomy that requires correction of glucometry levels and diarrhea. Conclusion: The von hipple lindau syndrome (VHL) is an autosomal dominant disease, in which the VHL protein is compromised, generating an increase in the production of vascular endothelial growth factors that predispose to the development of hemangioblastomas of the retina and the system central nervous system, renal cell carcinoma, pheochromocytoma, endolymphatic sac tumors, pancreatic lesions such as simple cysts, serous cystadenomas, neuroendocrine tumors and adenocarcinoma of the pancreas. Serous cystadenomas of the pancreas represent 10-15% of the cystic lesions of the pancreas, are characterized by a benign behavior and heterogeneous macroscopic findings, The association found in patients with VHL syndrome represents an average incidence of 50%, The risk of presenting diabetes mellitus is low despite the complete replacement of pancreatic parenchyma by cysts.