Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report

Background Neuroendocrine tumors (NETs) arise from neuroendocrine cells and are extremely rare in the biliary tract. Currently, there are no guidelines for the diagnosis and treatment of biliary NETs. We presented a case with NETs G1 of the hilar bile duct and the challenges for her treatment. Case presentation A 24-year-old woman was presented to our department with painless jaundice and pruritus, and the preoperative diagnosis was Bismuth type II hilar cholangiocarcinoma. She underwent Roux-en-Y hepaticojejunostomy with excision of the extrahepatic biliary tree and radical lymphadenectomy. Unexpectedly, postoperative pathological and immunohistochemical examination indicated a perihilar bile duct NETs G1 with the microscopic invasion of the resected right hepatic duct. Then the patient received 3 cycles of adjuvant chemotherapy (Gemcitabine and tegafur-gimeracil-oteracil potassium capsule). At present, this patient has been following up for 24 months without recurrence or disease progression. Conclusion We know little of biliary NETs because of its rarity. There are currently no guidelines for the diagnosis and treatment of biliary NETs. We reported a case of perihilar bile duct NETs G1 with R1 resection, as far as we know this is the first report. More information about biliary NETs should be registered.

A CADAVERIC STUDY ON VARIATIONS OF THE COMMON HEPATIC DUCT

Variations in the anatomy of extrahepatic biliary apparatus (EHBA) has been a subject of extended research due to its clinical implications. Cholecystectomy is the commonly performed abdominal surgeries and its safety requires the adequate appreciation of anatomical abnormalities of the extrahepatic biliary tree to decrease the morbidity and mortality of the surgery. Abnormalities of the major ducts and presence of accessory ducts give rise to preoperative difculties and postoperative complications. Background & objectives: To study the normal anatomy of common hepatic duct and its variations. With the Methods: aim of the above study, a prospective descriptive study was conducted on 55 specimens with reference to the ducts. Different parameters were used as union of right and left hepatic ducts and common hepatic duct measurements and looked for variations such as accessory hepatic ducts. Results & discussion: Extrahepatic union of right and left hepatic ducts seen in 98% cases and intrahepatic union in 2%. Length of common hepatic duct varied from 1.5 to 4.7 cm with an average of 2.9cm. Accessory hepatic ducts were seen in 7 cases (13%) in which 2 joined the common hepatic duct, 1 joined LHD and 3 were to the cystic duct. All the ndings of the ducts are to enlighte Conclusions: n the anatomical knowledge of the anatomists, general and laparoscopic surgeons, oncosurgeons and to the transplant surgeons which are abundantly useful.

Duodeno-pancreatic and extrahepatic biliary tree trauma: WSES-AAST guidelines

Duodeno-pancreatic and extrahepatic biliary tree injuries are rare in both adult and pediatric trauma patients, and due to their anatomical location, associated injuries are very common. Mortality is primarily related to associated injuries, but morbidity remains high even in isolated injuries. Optimal management of duodeno-bilio-pancreatic injuries is dictated primarily by hemodynamic stability, clinical presentation, and grade of injury. Endoscopic and percutaneous interventions have increased the ability to non-operatively manage these injuries. Late diagnosis and treatment are both associated to increased morbidity and mortality. Sequelae of late presentations of pancreatic injury and complications of severe pancreatic trauma are also increasingly addressed endoscopically and with interventional radiology procedures. However, for moderate and severe extrahepatic biliary and severe duodeno-pancreatic injuries, immediate operative intervention is preferred as associated injuries are frequent and commonly present with hemodynamic instability or peritonitis. The aim of this paper is to present the World Society of Emergency Surgery (WSES) and American Association for the Surgery of Trauma (AAST) duodenal, pancreatic, and extrahepatic biliary tree trauma management guidelines.

Exome Sequencing in Individuals with Isolated Biliary Atresia

Biliary atresia (BA) is a severe pediatric liver disease resulting in necroinflammatory obliteration of the extrahepatic biliary tree. BA presents within the first few months of life as either an isolated finding or with additional syndromic features. The etiology of isolated BA is unknown, with evidence for infectious, environmental, and genetic risk factors described. However, to date, there are no definitive causal genes identified for isolated BA in humans, and the question of whether single gene defects play a major role remains open. We performed exome-sequencing in 100 North American patients of European descent with isolated BA (including 30 parent-child trios) and considered several experimental designs to identify potentially deleterious protein-altering variants that may be involved in the disease. In a case-only analysis, we did not identify genes with variants shared among more than two probands, and burden tests of rare variants using a case-case control design did not yield significant results. In the trio analysis of 30 simplex families (patient and parent trios), we identified 66 de novo variants in 66 genes including a nonsense variant, p.(Cys30Ter), in the gene STIP1. STIP1 is a co-chaperone for the heat-shock protein, HSP90AA1, and has been shown to have diverse functions in yeast, flies and mammals, including stress-response.ConclusionOur results do not support the hypothesis that a simple genetic model is responsible for the majority of cases of isolated BA. Our finding of a de novo mutation in a candidate gene for BA (STIP1) linked to evolutionarily conserved stress responses suggests further exploration of how genetic susceptibility and environmental exposure interact to cause BA is warranted.

Anomalous anatomical variation in extrahepatic biliary tree and pancreas and its related vessels: a cadaveric study

Background: Congenital anamolies of extrahepatic biliary apparatus and pancreas have long been recognized and are of clinical importance because when present may surprise the surgeon during surgery and lead to iatrogenic injuries. Surgeries on extra-hepatic biliary apparatus and pancreas are regularly performed throughout the world. Thus insight into the normal anatomy and congenital variations will reduce complication and definitely improve outcome.Methods: Study was conducted in department of surgery GMC Bhopal and dissection was carried out in Department of Forensic Medicine on 100 cadavers with approval from ethical committee.Results: In 100 cases 70 were male and 30 female. The most common variation in extra hepatic biliary apparatus was short cystic duct was found in 6% cases then formation of common hepatic duct by union of right hepatic duct and left hepatic duct was intrahepatic in 3% cases. There was low insertion of cystic duct with common hepatic duct in 1% case. Cystic artery originating from left hepatic artery in 1% case, in 1% case cystic artery was anterior to common hepatic duct. In Pancreas anterior arterial arcade was absent in 2% cases and its origin varied in 2% case. Posterior pancreatic arcade absent in 1% cases and variation in origin was present in 1% case. The variation in pancreatic duct course was present in 22% cases.Conclusions: Thus significant variation was seen and it could definitely be helpful to hepatobiliary, laproscopic surgeons, radiologist and will further contribute to literature on variation of extrahepatic biliary apparatus and pancreas and its related vessels.