von Hippel (1) and A Lindau (2) early in the 20th century It is

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. However, approximately 20% of these mutations occur de novo (3). It is typically 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 www.selleckchem.com/products/ganetespib-sta-9090.html 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 http://www.selleckchem.com/products/pacritinib-sb1518.html hepatoduodenal ligament would render this surgical option unnecessarily risky, if not unwantedly Cilengitide 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.

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