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.

Combined malformation of the biliary tract

Anomalies in the biliary tract development are a relatively rare pathology in the practice of a pediatric surgeon. A combination of two or more defects of the biliary tract is even less common. The article presents literature data and own clinical observation of a child with a combination of a cyst of the common bile, or hepatic, duct (common bile duct) and an additional bile duct. The anatomical structure of the biliary tract was identified before surgery using magnetic resonance cholangiography. Based on the analysis of literature data and our own observation, we propose an algorithm for diagnostic measures in children with malformations of the biliary tract, which helps to avoid intraoperative injuries.

Recurrent intraductal papillary neoplasm of the bile duct due to intraductal dissemination: a case report and literature review

Background Intraductal papillary neoplasm of the bile duct (IPNB) is a subtype of biliary tumor. The 5-year survival rate of patients with IPNB who underwent curative resection is 81%. However, IPNB is known to often recur in other parts of the bile duct. Nevertheless, its mechanism remains poorly understood. Herein, we report the case of a patient with recurrent IPNB, which was considered to be attributed to intraductal dissemination in the common bile duct at 12 months after curative resection. We also made a review of the existing literature. Case presentation A 69-year-old man was referred to our hospital for the evaluation and dilation of an intrahepatic bile duct (IHBD) mass. Computed tomography (CT) findings confirmed a mass in the left hepatic duct. Left trisectionectomy, extrahepatic bile duct resection with biliary reconstruction, and regional lymph node dissection were performed. Intraoperative examination of the resection margin at the common bile duct and posterior segmental branch of the hepatic duct was negative for the presence of malignant cells. Histologically, the tumor showed intraductal papillary growth of the mucinous epithelium and was diagnosed as non-invasive IPNB. It had a papillary structure with atypical epithelial cells lined up along the neoplastic fibrovascular stalks. Immunohistochemically, this was as a gastric-type lesion. At 12 postoperative months, CT revealed a 1.5-cm mass in the lower remnant common bile duct. We performed subtotal stomach-preserving pancreaticoduodenectomy. The tumor exhibited papillary growth and was microscopically and immunohistochemically similar to the first tumor. At approximately 16 months after the patient’s second discharge, CT showed an abdominal mass at the superior mesenteric plexus, which was diagnosed as recurrent IPNB. Chemotherapy is ongoing, and the patient is still alive. In this case, as described in many previous reports, IPNB recurred below the primary lesion in the bile duct. Conclusion Based on our review of previous reports on IPNB recurrence, intraductal dissemination was considered one of the mechanisms underlying recurrence after multicentric development. Considering the high frequency and oncological conversion of recurrence in IPNB, regular follow-up examination is essential to achieve better prognosis in patients with recurrent IPNB.

Diagnosis of a single gallbladder with double cystic ducts and dominant accessory duct draining into the right hepatic duct: a case report

Biliary anomalies are a high risk for biliary injury during surgery, and although a biliary anomaly is occasionally encountered, variations in cystic ducts are rare. A preoperative diagnosis is highly valuable in facilitating surgical procedures and avoiding surgical complications. Herein, the case of a 67-year-old female patient with acute cholecystitis, in which preoperative fluoroscopic cholangiography clearly demonstrated a single gallbladder with double cystic ducts, is presented. The accessory duct was found to be dominant, draining into the otherwise normal right intrahepatic bile duct, and laparoscopic cholecystectomy was performed smoothly and successfully. Fluoroscopic cholangiography is a powerful tool that may clearly depict the anomaly of a single gallbladder with double cystic ducts. Through appropriate preoperative knowledge and demonstration of this biliary anomaly in the present case, laparoscopic cholecystectomy was safely performed, and the patient was symptom-free at the 3-year follow-up assessment.

Klatskin-Mimicking Lesions

Altemeier-Klatskin tumor is a perihilar cholangiocarcinoma that occurs within 2 cm of the confluence of the right and left hepatic duct at the hepatic hilum and accounts for 50–70% of all cholangiocarcinomas cases. Although imaging techniques have come very far today, this entity can still be very challenging to diagnose as there are many lesions that can mimic Klatskin tumor. In this review, we will present the most common Klatskin-mimicking lesions.

An Unusual Cause of Cholestasis in an Infant: Biliary Atresia Type IIB

Biliary atresia (BA) is a progressive destructive cholangiopathy of unknown etiology that presents in early infancy. It has a worldwide frequency of 1:8,000–1:15,000 and is common in Asia than in the west. Based on the level at which the lumen of the extrahepatic duct is obliterated, BA is classified into three types. Type III is the commonest (∼85%) type and has the most proximal level of obstruction in the porta hepatis, while type II in which the atresia is at the level of the common hepatic duct, is the least common (∼2.5%) and has been rarely reported. Here, we report the imaging features of an infant with type IIB biliary atresia.

Metastatic Neoplasms of the Large Bile Ducts- A Clinicopathological Study

Introduction/Objective Biliary strictures are often considered malignant until proven otherwise. While the majority of malignant biliary strictures represent a primary neoplasm, secondary involvement by metastasis also rarely occurs. Primary cholangiocarcinoma and metastatic disease have different treatment considerations and likely different prognoses. The aim of this study is to look at the clinico-pathological characteristics of metastatic neoplasms of the bile duct. Methods/Case Report We retrospectively searched the pathology archives for biliary biopsies between 1991-2020. Patients with primary biliary, gallbladder, pancreatic, ampullary and hepatic malignancies and all cases of lymphoma were excluded from the study. A total of 20 cases were included. Results (if a Case Study enter NA) The median age of the patients was 63 years with a M:F ratio of 1.9:1. The biopsies were taken from the common bile duct (n=17), common hepatic duct (n=2) and left hepatic duct (n=1). 8 patients had synchronous and 12 had metachronous presentation. The overall median interval between the bile duct metastasis and primary was 18 months (Range: 0-100 months) for all patients and 33 months for metachronous cases. For 13 tumors, the primary site of origin was in the gastrointestinal tract (colon: 7; stomach: 4; anal canal: 1; gastro-esophageal junction: 1). Other primary sites included breast (3 cases), lung, endometrium and adrenal (1 each). One case presented with metastatic melanoma with an occult primary. Adenocarcinoma was the most common histological subtype seen in 17 cases. Other histological subtypes were squamous cell carcinoma, adrenocortical carcinoma and melanoma. Conclusion Secondary involvement of the bile duct by metastasis is rare. Most cases are metastasis from the lumenal gastrointestinal tract, with colon being the most common primary site. They are more likely to have a metachronous presentation with rare instances of bile duct metastasis as the first presentation. Awareness of secondary involvement of the biliary tree by metastasis is important as they can have prognostic and therapeutic significance.