von Hippel (1) and A. Lindau (2) early in the 20th century. It is a rare highly penetrant autosomal dominant genetic predisposition to malignant and benign tumors, emanating from over 1,000 possible mutations in the VHL tumor suppressor gene on chromosome 3p25. how to order However, approximately 20% of these mutations occur de novo (3). It is typically sellekchem associated with central nervous system hemangioblastomas, clear renal cell carcinomas, cystadenomas, and neuro-endocrine tumors depending on the sub-type (4). Patients with VHL type I usually manifest hemangioblastomas but rarely present with clear renal cell carcinoma or pheochromocytoma. On the Inhibitors,research,lifescience,medical other hand, patients with VHL type II comprises

sub-types A, B, and C, which predispose to the development of hemangioblastoma; hemangioblastomas and clear renal cell carcinoma; and pheochromocytomas; respectively (4). The rarely observed VHL type III is associated with Chuvash polycythemia. Pancreatic serous cystadenomas Inhibitors,research,lifescience,medical are entail a relatively rare VHL presentation, affecting only ~10% of patients (5). Further rarely, the above

mentioned patient sub-population develops Inhibitors,research,lifescience,medical hepato-biliary obstruction requiring decompression (6). In the presented case, the pancreatic serous cystadenomas (Figure 1) was unresectable as per the extent of the disease and the concomitant portal hypertension. Yet, a suitable palliative alternative was de rigueur. Percutaneous cholecytostomy tube (PTC) would transiently palliate hepato-billiary, but would not alleviate gastric outlet obstruction. PTC is inconvenient in a functional patient with favorable prognosis. Duodenal stent option was limited by the distorted anatomy and would not resolve the biliary obstruction. Ideally, this patient would require

a choledocho- (or haepatico-) jejunostomy and a gastro-jejunostomy Inhibitors,research,lifescience,medical fashioned on a single limb or through a Roux-en-Y reconstructive bypass. Nevertheless, extensive portal-hypertension Inhibitors,research,lifescience,medical secondary to occlusion of the portal vein and the subsequent development of varices at the level of the hepatoduodenal ligament would render this surgical option unnecessarily risky, if not unwantedly GSK-3 morbid. Along the same line, any laparoscopic approach would be discouraging. As a result, a cholecysto-jejunostomy (in addition to a gastro-jejunostomy) was performed. Currently, this procedure turned into a mere historical curiosity—it is performed only as a last resort, and most often in the developing world where resources are scarce, expertise is scant, and patients generally present with advanced disease (7). However, this procedure constitutes a safe and effective last-resort in the hepato-billiary armamentary. Undoubtedly, the surgical procedure described above does not treat portal hypertension. But since the patient remains asymptomatic, neither a surgical shunt nor a trans-jugular intra-hepatic systemic shunt is currently indicated. It is hard to explain the uncontrolled hyperglycemia experienced by the patient.