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We describe a case of a 47-year-old male patient with initially unresectable intrahepatic cholangiocarcinoma of the right liver lobe with tumor thrombi extending from the right bile duct to the common and left bile ducts. Conventional chemotherapy with gemcitabine and cisplatin for 19 months resulted in progressive disease. Subsequently, a comprehensive genome profile revealed fibroblast growth factor receptor 2 rearrangement, and hence, pemigatinib administration was initiated. After 6 months of pemigatinib therapy, significant shrinking of the tumor and disappearance of the tumor thrombi in the common and left bile duct were observed. Subsequently, the patient underwent conversion surgery, resulting in successful radical resection of the tumor. The patient has been disease-free for 7 months. [ABSTRACT FROM AUTHOR]

Background/Aims Endoscopic ultrasound-guided tissue acquisition (EUS-TA) is a standard diagnostic method for biliary tract cancer (BTC), and samples obtained in this manner may be used for comprehensive genomic profiling (CGP). This study evaluated the utility of EUS-TA for CGP in a clinical setting and determined the factors associated with the adequacy of CGP in patients with BTC. Methods CGP was attempted for 105 samples from 94 patients with BTC at the Aichi Cancer Center, Japan, from October 2019 to April 2022. Results Overall, 77.1% (81/105) of the samples were adequate for CGP. For 22-G or 19-G fine-needle biopsy (FNB), the sample adequacy was 85.7% (36/42), which was similar to that of surgical specimens (94%, p=0.45). Univariate analysis revealed that 22-G or larger FNB needle usage (86%, p=0.003), the target primary lesions (88%, p=0.015), a target size ≥30 mm (100%, p=0.0013), and number of punctures (90%, p=0.016) were significantly positively associated with CGP sample adequacy. Conclusions EUS-TA is useful for CGP tissue sampling in patients with BTC. In particular, the use of 22-G or larger FNB needles may allow for specimen adequacy comparable to that of surgical specimens.

Background/Aims: Endoscopic ultrasound-guided tissue acquisition (EUS-TA) is a standard diagnostic method for biliary tract cancer (BTC), and samples obtained in this manner may be used for comprehensive genomic profiling (CGP). This study evaluated the utility of EUS-TA for CGP in a clinical setting and determined the factors associated with the adequacy of CGP in patients with BTC. Methods: CGP was attempted for 105 samples from 94 patients with BTC at the Aichi Cancer Center, Japan, from October 2019 to April 2022. Results: Overall, 77.1% (81/105) of the samples were adequate for CGP. For 22-G or 19-G fine-needle biopsy (FNB), the sample adequacy was 85.7% (36/42), which was similar to that of surgical specimens (94%, p=0.45). Univariate analysis revealed that 22-G or larger FNB needle usage (86%, p=0.003), the target primary lesions (88%, p=0.015), a target size ≥30 mm (100%, p=0.0013), and number of punctures (90%, p=0.016) were significantly positively associated with CGP sample adequacy. Conclusions: EUS-TA is useful for CGP tissue sampling in patients with BTC. In particular, the use of 22-G or larger FNB needles may allow for specimen adequacy comparable to that of surgical specimens. [ABSTRACT FROM AUTHOR]

Abstract Resection is the only potential curative treatment for perihilar cholangiocarcinoma (PHC); however, complete resection is often technically challenging due to the anatomical location. Various innovative approaches and procedures were invented to circumvent this limitation but the rates of postoperative morbidity (20%–78%) and mortality (2%–15%) are still high. In patients diagnosed with resectable PHC, deliberate and coordinated preoperative workup and optimization of the patient and future liver remnant are crucial. Biliary drainage is recommended to relieve obstructive jaundice and optimize the clinical condition before liver resection. Biliary drainage for PHC can be performed either by endoscopic biliary drainage or percutaneous transhepatic biliary drainage. To date there is no consensus about which method is preferred. The volumetric assessment of the future remnant liver volume and optimization mainly using portal vein embolization is the gold standard in the management of the risk to develop post hepatectomy liver failure. The improvement of systemic chemotherapy has contributed to prolong the survival not only in patients with unresectable PHC but also in patients undergoing curative surgery. In this article, we review the literature and discuss the current surgical treatment of PHC.

Abstract Aim Neoadjuvant chemoradiotherapy may improve survival in patients with advanced cholangiocarcinoma. This Phase I study aimed to determine the recommended dose of neoadjuvant chemoradiotherapy and decide whether to move to a Phase II study. Methods Patients diagnosed with resectable stage II–IVa cholangiocarcinoma were administered cisplatin (40 [level 0], 50 [level 1 as starting dose], or 60 [level 2] mg/m2), 80 mg/m2 of S‐1, and 50.4 Gy of external beam radiation. The recommended dose was defined as a dose one‐step lower than the maximum‐tolerated dose, which was defined when dose‐limiting toxicity was observed in three or more of the six patients. Results Twelve patients were eligible from November 2012 to May 2016. Ten patients had perihilar cholangiocarcinoma and two patients had distal cholangiocarcinoma. Dose‐limiting toxicity was observed in one of the first six patients at level 1 and two of the next six patients at level 2; thus, the maximum‐tolerated dose was not determined even at level 2 and the recommended dose was determined as level 2. Four patients had partial response, four patients had stable disease, and two patients had progression of disease because of liver metastases. Finally, nine patients underwent radical surgery and seven cases achieved R0 resection. However, five cases suffered biliary leakage and one suffered intrahospital death due to rupture of the hepatic artery. Conclusion We determined the recommended dose of neoadjuvant chemoradiotherapy for resectable cholangiocarcinoma. However, we terminated the trial due to a high incidence of morbidity and unexpected mortality.

Resection is the only potential curative treatment for perihilar cholangiocarcinoma (PHC); however, complete resection is often technically challenging due to the anatomical location. Various innovative approaches and procedures were invented to circumvent this limitation but the rates of postoperative morbidity (20%–78%) and mortality (2%–15%) are still high. In patients diagnosed with resectable PHC, deliberate and coordinated preoperative workup and optimization of the patient and future liver remnant are crucial. Biliary drainage is recommended to relieve obstructive jaundice and optimize the clinical condition before liver resection. Biliary drainage for PHC can be performed either by endoscopic biliary drainage or percutaneous transhepatic biliary drainage. To date there is no consensus about which method is preferred. The volumetric assessment of the future remnant liver volume and optimization mainly using portal vein embolization is the gold standard in the management of the risk to develop post hepatectomy liver failure. The improvement of systemic chemotherapy has contributed to prolong the survival not only in patients with unresectable PHC but also in patients undergoing curative surgery. In this article, we review the literature and discuss the current surgical treatment of PHC. [ABSTRACT FROM AUTHOR]

Aim: Neoadjuvant chemoradiotherapy may improve survival in patients with advanced cholangiocarcinoma. This Phase I study aimed to determine the recommended dose of neoadjuvant chemoradiotherapy and decide whether to move to a Phase II study. Methods: Patients diagnosed with resectable stage II–IVa cholangiocarcinoma were administered cisplatin (40 [level 0], 50 [level 1 as starting dose], or 60 [level 2] mg/m2), 80 mg/m2 of S‐1, and 50.4 Gy of external beam radiation. The recommended dose was defined as a dose one‐step lower than the maximum‐tolerated dose, which was defined when dose‐limiting toxicity was observed in three or more of the six patients. Results: Twelve patients were eligible from November 2012 to May 2016. Ten patients had perihilar cholangiocarcinoma and two patients had distal cholangiocarcinoma. Dose‐limiting toxicity was observed in one of the first six patients at level 1 and two of the next six patients at level 2; thus, the maximum‐tolerated dose was not determined even at level 2 and the recommended dose was determined as level 2. Four patients had partial response, four patients had stable disease, and two patients had progression of disease because of liver metastases. Finally, nine patients underwent radical surgery and seven cases achieved R0 resection. However, five cases suffered biliary leakage and one suffered intrahospital death due to rupture of the hepatic artery. Conclusion: We determined the recommended dose of neoadjuvant chemoradiotherapy for resectable cholangiocarcinoma. However, we terminated the trial due to a high incidence of morbidity and unexpected mortality. [ABSTRACT FROM AUTHOR]

Cholangiocarcinoma (CCA) is a heterogeneous group of neoplasms of the bile ducts and represents the second most common hepatic cancer after hepatocellular carcinoma; it is sub-classified as intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA), the latter comprising both perihilar cholangiocarcinoma (pCCA or Klatskin tumor), and distal cholangiocarcinoma (dCCA). The global incidence of CCA has increased worldwide in recent decades. Chronic inflammation of biliary epithelium and bile stasis represent the main risk factors shared by all CCA sub-types. When feasible, liver resection is the treatment of choice for CCA, followed by systemic chemotherapy with capecitabine. Liver transplants represent a treatment option in patients with very early iCCA, in referral centers only. CCA diagnosis is often performed at an advanced stage when CCA is unresectable. In this setting, systemic chemotherapy with gemcitabine and cisplatin represents the first treatment option, but the prognosis remains poor. In order to ameliorate patients’ survival, new drugs have been studied in the last few years. Target therapies are directed against different molecules, which are altered in CCA cells. These therapies have been studied as second-line therapy, alone or in combination with chemotherapy. In the same setting, the immune checkpoints inhibitors targeting programmed death 1 (PD-1), programmed death-ligand 1 (PD-L1), cytotoxic Tlymphocyte antigen-4 (CTLA-4), have been proposed, as well as cancer vaccines and adoptive cell therapy (ACT). These experimental treatments showed promising results and have been proposed as second- or third-line treatment, alone or in combination with chemotherapy or target therapies. [ABSTRACT FROM AUTHOR]

The diagnosis of biliary strictures in clinical practice can be challenging. Discriminating between benign and malignant biliary strictures is important to prevent the morbidity and mortality associated with incorrect diagnoses. Missing a malignant biliary stricture may delay surgery, resulting in poor prognostic outcomes. Conversely, it has been demonstrated that approximately 20% of patients who undergo surgery for suspected biliary malignancies have a benign etiology on histopathology. Traditional tissue sampling using endoscopic retrograde cholangiography does not always produce a definitive diagnosis, with a considerable proportion of cases remaining as indeterminate biliary strictures. Recent advances in endoscopic techniques have the potential to improve the diagnostic and prognostic accuracy of biliary strictures.

Cholangiocarcinoma (CCA) is a heterogeneous group of neoplasms of the bile ducts and represents the second most common hepatic cancer after hepatocellular carcinoma; it is sub-classified as intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA), the latter comprising both perihilar cholangiocarcinoma (pCCA or Klatskin tumor), and distal cholangiocarcinoma (dCCA).The global incidence of CCA has increased worldwide in recent decades. Chronic inflammation of biliary epithelium and bile stasis represent the main risk factors shared by all CCA sub-types.When feasible, liver resection is the treatment of choice for CCA, followed by systemic chemotherapy with capecitabine. Liver transplants represent a treatment option in patients with very early iCCA, in referral centers only. CCA diagnosis is often performed at an advanced stage when CCA is unresectable. In this setting, systemic chemotherapy with gemcitabine and cisplatin represents the first treatment option, but the prognosis remains poor.In order to ameliorate patients’ survival, new drugs have been studied in the last few years. Target therapies are directed against different molecules, which are altered in CCA cells. These therapies have been studied as second-line therapy, alone or in combination with chemotherapy. In the same setting, the immune checkpoints inhibitors targeting programmed death 1 (PD-1), programmed death-ligand 1 (PD-L1), cytotoxic T-lymphocyte antigen-4 (CTLA-4), have been proposed, as well as cancer vaccines and adoptive cell therapy (ACT). These experimental treatments showed promising results and have been proposed as second- or third-line treatment, alone or in combination with chemotherapy or target therapies.

The diagnosis of biliary strictures in clinical practice can be challenging. Discriminating between benign and malignant biliary strictures is important to prevent the morbidity and mortality associated with incorrect diagnoses. Missing a malignant biliary stricture may delay surgery, resulting in poor prognostic outcomes. Conversely, it has been demonstrated that approximately 20% of patients who undergo surgery for suspected biliary malignancies have a benign etiology on histopathology. Traditional tissue sampling using endoscopic retrograde cholangiography does not always produce a definitive diagnosis, with a considerable proportion of cases remaining as indeterminate biliary strictures. Recent advances in endoscopic techniques have the potential to improve the diagnostic and prognostic accuracy of biliary strictures. [ABSTRACT FROM AUTHOR]

Background: The prevalent location and incidence of intraductal papillary neoplasm of the bile duct (IPNB) and invasive carcinoma associated with them have varied markedly among studies due to differences in diagnostic criteria and tumor location. Methods: IPNBs were classified into two types: Type 1 IPNB, being histologically similar to intraductal papillary mucinous neoplasm of the pancreas, and Type 2 IPNB, having a more complex histological architecture with irregular papillary branching or foci of solid‐tubular components. Medical data were evaluated. Results: Among 694 IPNB patients, 520 and 174 had Type 1 and Type 2, respectively. The levels of AST, ALT, ALP, T. Bil, and CEA were significantly higher in patients with Type 2 than in those with Type 1. Type 1 IPNB was more frequently located in the intrahepatic bile duct than Type 2, whereas Type 2 was more frequently located in the distal bile duct than Type 1 IPNB (P < 0.001). There were significant differences in 5‐year cumulative survival rates (75.2% vs 50.9%; P < 0.0001) and 5‐year cumulative disease‐free survival rates (64.1% vs 35.3%; P < 0.0001) between the two groups. Conclusion: Type 1 and Type 2 IPNBs differ in their clinicopathological features and prognosis. This classification may help to further understand IPNB. [ABSTRACT FROM AUTHOR]

Primary sclerosing cholangitis (PSC) is a chronic, fibroinflammatory, progressive cholangiopathy. In a clinically significant proportion of patients, the disease course of PSC is punctuated by carcinogenesis, namely cholangiocarcinoma, gallbladder carcinoma, hepatocellular carcinoma, and/or colorectal carcinoma. Indeed, malignancy is arguably the most consequential sequela and the cause of nearly 50% of deaths in patients with PSC. This statistic is multifactorial, relating partly to the premalignant nature of PSC, challenges in diagnosis due to obscuration of cancer by the inflammation and fibrosis inherent to PSC, and the unpredictability of which type of cancer will develop in PSC and when. Here, in the second of a 2-part series, we review cancer risk, prevention, and surveillance in patients with PSC. We also discuss potential cancer surveillance strategies in PSC and, where evidence is limited, make pragmatic recommendations based on current data and expert opinion. [ABSTRACT FROM AUTHOR]

Biliary cystadenoma is a rare tumor of the biliary tree and a rare cause of obstructive jaundice. Most are intrahepatic, and pure extrahepatic biliary cystadenoma is less common. Cases are more common in women. Unless suspected, diagnosis of extrahepatic biliary cystadenoma is often delayed. Here, we report the case of a young woman with extrahepatic biliary cystadenoma who presented at Raja Isteri Pengiran Anak Saleha Hospital with obstructive jaundice initially thought to be due to a large biliary stone based on the endoscopic cholangiogram image. She was successfully managed with resection of the cystadenoma.

Background/Aims: Palliative chemotherapy is the main treatment for advanced biliary tract cancer (BTC). However, there is a lack of established second-line chemotherapy to treat disease progression after first-line chemotherapy. We examined combination therapy with capecitabine and cisplatin for advanced BTC as a second-line regimen. Methods: We analyzed the medical records of 40 patients diagnosed with BTC who received palliative second-line chemotherapy with capecitabine and cisplatin. Results: The median overall survival from the start of second-line chemotherapy was 6.3 months. The median overall survival from diagnosis was 17.9 months. The median progression-free survival during second-line chemotherapy was 2.3 months. Nine (30%) patients experienced adverse events of grade ≥ 3. Eastern Cooperative Oncology Group performance score was an independent predictor of adverse events. Conclusions: Combination therapy with capecitabine and cisplatin may be an option for second-line chemotherapy in some of patients with advanced BTC. [ABSTRACT FROM AUTHOR]

Cholangiocarcinoma is an aggressive tumor of the hepatic biliary system and it commonly spreads to the regional lymph nodes, liver and lungs. However, bone metastasis from cholangiocarcinoma is rare compared with other tumors. We herein present the case of a 61-year-old Asian woman who presented with pain in the right scapular area. Magnetic resonance imaging revealed bone destruction and an adjacent soft tissue mass at the right scapula. The findings on computed tomography imaging were compatible with cholangiocarcinoma. Bone biopsy was performed and the diagnosis of cholangiocarcinoma with bone metastasis was confirmed. The survival time was 10 months, despite administration of palliative radiotherapy and chemotherapy. Therefore, bone metastasis from cholangiocarcinoma should be considered as a differential diagnosis in patients who present with an osteolytic bone lesion and a liver mass. [ABSTRACT FROM AUTHOR]

Background: The placement of a self-expandable metallic stent (SEMS) is a widely used nonsurgical treatment method in patients with unresectable malignant biliary obstructions but SEMS is susceptible to occlusion by tumor ingrowth or overgrowth. Aim: The efficacy and safety of a metallic stent covered with a paclitaxel-incorporated membrane (MSCPM) in which paclitaxel provided an antitumoral effect was compared prospectively with those of a covered metal stent (CMS) in patients with malignant biliary obstructions. Methods: Patients with unresectable distal malignant biliary obstructions ( n = 106) were prospectively enrolled in this study at multiple treatment centers. A MSCPM was inserted endoscopically in 60 patients, and a CMS was inserted in 46 patients. Patients underwent systemic chemotherapy regimens alternatively according to disease characteristics. Results: The two groups did not differ significantly in mean age, male to female ratio, or mean follow-up period. Stent occlusion due to tumor ingrowth occurred in 12 patients who received MSCPMs and in eight patients who received CMSs. Stent patency and survival time did not differ significantly between the two groups ( p = 0.116, 0.981). Chemotherapy had no influence on stent patency, but gemcitabine-based chemotherapy was a significant prognostic factor for survival time ( p = 0.012). Complications, including cholangitis and pancreatitis, were found to be acceptable in both groups. Conclusions: Although the use of a MSCPM produced no significant differences in stent patency or patient survival in patients with malignant biliary obstructions compared with the use of a CMS, this study demonstrated that MSCPM can be used safely in humans. [ABSTRACT FROM AUTHOR]

Introduction. Concomitant biliary and duodenal obstructions are not uncommon complications in patients with gastroduodenal or pancreatobiliary malignancies. Alleviation of obstruction is very important for the palliation of inoperable patients. We studied the clinical outcomes of combined biliary and duodenal stenting. Methods. Between January 2003 and January 2010, the records of 24 patients who underwent biliary and duodenal stent placement due to inoperable malignant biliary and duodenal obstruction were reviewed retrospectively. Results. Of the 24 patients, a duodenal stent was placed after biliary stenting in 23 patients and a biliary stent was placed after duodenal stenting in one patient. Biliary stents were placed endoscopically (33 cases) or percutaneously (14 cases). Duodenal stents were placed endoscopically by fluoroscopic guidance in all patients. Oral feeding was possible at a mean of 2.7 ± 1.2 days (range, 1-6 days) after duodenal stenting. Acute pancreatitis and acute cholangitis developed in three patients and one patient, respectively, as early complications after biliary stenting. Biliary stent occlusion was developed in 12 patients and was treated successfully by stent reinsertion. As complications of duodenal stent, one case of stent migration and five cases of stent occlusion developed. Median survival after initial bilioduodenal stenting was 195.5 days (range, 21-725 days). Stent patency was well maintained in 83.3% of patients after combined stent placements while patients were alive. Conclusion. Combined biliary and duodenal stenting seems to be safe and effective in palliation of inoperable malignant biliary and duodenal obstruction. [ABSTRACT FROM AUTHOR]

Background: The clinical effect of stent treatment has been evaluated by mainly physicians; only a limited number of prospective studies have used patient-reported outcomes for this purpose. The aim of this work was to study the clinical effect of self-expanding metal stents in treatment of malignant gastrointestinal obstructions, as evaluated by patient-reported outcomes, and compare the rating of the treatment effect by patients and physicians. Methods: Between November 2006 and April 2008, 273 patients treated with SEMS for malignant GI and biliary obstructions were recruited from nine Norwegian hospitals. Patients and physicians assessed symptoms independently at the time of treatment and after 2 weeks using the European Organisation for Research and Treatment of Cancer (EORTC) QLQ-C30 questionnaire supplemented with specific questions related to obstruction. Results: A total of 162 patients (99 males; median age = 72 years) completed both assessments and were included in the study. A significant improvement in the mean global health score was observed after 2 weeks (from 9 to 18 on a 0-100 scale, P < 0.03) for all stent locations. Both patients and physicians reported a significant reduction in all obstruction-related symptoms (>20 on the 0-100 scale, P < 0.006) after SEMS treatment. The physicians reported a larger mean improvement in symptoms than did the patients, mainly because they reported more severe symptoms before treatment. Conclusion: SEMS treatment is effective in relieving symptoms of malignant GI and biliary obstruction, as reported by patients and physicians. The physicians, however, reported a larger reduction in obstructive symptoms than did the patients. A prospective assessment of patient-reported outcomes is important in evaluating SEMS treatment. [ABSTRACT FROM AUTHOR]

Aim: To compare the efficacy of plastic stents in the treatment of distal and proximal stricture of biliary tract neoplasm’s. Methods: From January 2002 to February 2009, 70 patients (37 males, 33 females) with non surgically resectable malignant biliary obstruction who received plastic stent implantation were reviewed retrospectively. The stents were inserted by endoscopy. The endoscopist implants one or more stents if he considers this necessary. The end points of the study were stent occlusion and patient death. Results: The mean time of stent patency was 81 ± 75 days in the group of proximal stricture (group 1) and 130 ± 68 days in the group of distal stricture (group 2), (p 0.40, with CI 95% 0.47-1.37). The mean survival time was 126 days in group 1 and 159 days in group 2. There was not a significant difference between the two groups. Conclusion: Plastic stent implantation is a feasible, palliative method for unresectable malignant biliary obstruction. The overall survival and obstruction with the use of plastic stent in patients with distal stenoses compared with proximal obstruction was not significantly different in our study. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Collision cancers are malignancies in the same organ or anatomical site that comprises at least two different tumor components, with no mixed or transitional area between two components. Collision cancers are very rare in the pancreas and periampullary region. The aim of this study was to analyze the clinical and pathological features and prognosis of collision cancer in the pancreas and periampullary region. METHODS: Patients with collision cancers of the pancreas and periampullary region ( n= 10) who had undergone radical surgery were retrospectively studied. Their clinical and pathological features were summarized and the prognostic data were compared with patients with pancreatic adenocarcinomas who underwent radical surgery ( n= 87) and with patients with pancreatic or periampullary malignancies who underwent palliative surgery ( n= 89). RESULTS: Compared with other cancers at these sites, collision cancer presents no specific clinical features. However, the median survival period of patients with such malignancies was only 10.0 months, which was much less than those with pancreatic adenocarcinomas who underwent radical surgery (27.0 months) and those who received a palliative operation (20.9 months) only. CONCLUSION: Collision cancers of the pancreas and periampullary region are difficult to diagnose preoperatively. Their prognosis is poor even after radical resection and adjuvant chemotherapy were given. [ABSTRACT FROM AUTHOR]

Background: Overexpression of signaling proteins including epidermal growth factor receptor (EGFR), Akt, mitogen activated protein kinase (MAPK) and cyclooxygenase-2 (COX-2) occurs in cholangiocarcinoma cell lines. However, the prognostic value of these markers is unknown. No prior study correlated the expression of these signaling proteins with clinical outcome. Further, co-expression of these proteins has not been reported. Co-expression may reflect cross-talk between signaling pathways. The aim of this clinicopathological study was to investigate the overexpression and co-expression of EGFR and related signaling proteins in cholangiocarcinoma and explore their relationship to clinical outcome. Methods: Twenty-four consecutive cases of cholangiocarcinoma treated from 1996 to 2002 at Roswell Park Cancer Institute were included. Immunohistochemical staining of paraffin-embedded tissue sections was performed using antibodies against Akt, p-Akt, MAPK, p-MAPK, COX-2, EGFR and p-EGFR. Two pathologists independently scored the protein expression. Results: Cyclooxygenase-2, Akt, and p-MAPK were commonly expressed in biliary cancers (100%, 96% and 87% of malignant cells, respectively). EGFR (60%) and p-EGFR (22%) overexpression was also detected. There was a significant association between EGFR and p-EGFR ( P = 0.027) and between Akt and p-Akt ( P = 0.017) expression in tumor tissue. A noteworthy association was shown between MAPK and p-Akt ( P = 0.054). Multivariate analysis using the Cox proportional hazard model identified the use of chemotherapy (hazard ratio [HR] = 0.039, P = 0.0002), radiation (HR = 0.176, P = 0.0441) and Akt expression (HR = 0.139, P = 0.006) as the best predictors of overall prognosis. Conclusion: Epidermal growth factor receptor signaling intermediates are commonly expressed in cholangiocarcinoma. Expression of Akt and use of systemic chemotherapy or radiation may correlate with improved survival. [ABSTRACT FROM AUTHOR]

Objective: To clarify the role of chemotherapy for advanced gallbladder cancer (GBC). Methods: We reviewed 89 GBC patients: 21 admitted before 1997 were treated with a combination of cisplatin, epirubicin, and 5-fluorouracil (CEF); 25, admitted subsequently, received a combination of 5-fluorouracil, doxorubicin and mitomycin (FAM), and the remaining 43, ineligible for these trials, received supportive care. We investigated the relation between pretreatment clinical variables and long-term survival in these 89 subjects, and analyzed whether chemotherapy could favor longer survival. Results: There were no significant differences in survival time between the chemotherapy groups, whereas the response rate to the CEF regimen was 4-fold higher than to the FAM regimen (32 vs. 8%). Subgroup analysis suggested that chemotherapy favored longer survival in patients with a performance status (PS) of 0 or 1, but not in patients with a PS of 2. Cox regression analysis suggested a significant hazard reduction by chemotherapy in patients with a PS of 0 or 1, but not in patients with a PS of 2. Conclusions: GBC patients with poor PS should not be treated with chemotherapy at present. It is essential to design good clinical trials and develop more effective chemotherapy regimens. Copyright © 2004 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

To review the experience with endoscopic palliative treatment for malignant obstructive jaundice (MOJ) in 929 patients. 929 patients (598 males and 331 females) underwent 1215 endoscopic palliative drainages for MOJ in our hospital. Tumor obstruction occurred in the distal common bile duct (CBD) (263 patients), the middle CBD (43), and the hilum (909). The mean bilirubin level was 284.3 μmol/L (range 26–810). Of the 1215 drainages, 599 were performed by stenting with plastic endoprosthesis, 385 by naso-biliary catheterization, 167 by expandable metal stents, 39 by combined drainage method, 19 by tumor sphincterotomy and 6 by endoscopic fistulostomy. The successful rate of endoscopic procedure was 94.3%. The jaundice symptom was improved in 81.8% of the patients with a significant reduction of serum bilirubin in 64.7%. The complication after treatment occurred in 23 cases (2.6%), including cholangitis (23 cases), pancreatitis (8 cases), and bleeding (one case), and 3 cases were dead (0.2%). The median survival time of all patients was 14 months and life time analysis showed a life expectancy of 75.9%, 44.0% and 25.2% at 1, 2 and 3 years respectively. In the patients with MOJ secondary to pancreatobiliary malignancy, successful endoscopic drainage provides adequate relief of biliary obstruction and is associated with low morbidity and mortality. Endoscopic interventional treatment may be considered as an alternative of palliative biliary operation for the late stage of pancreatic and biliary malignancies. [ABSTRACT FROM AUTHOR]

Biliary cystadenoma is a rare tumor of the biliary tree and a rare cause of obstructive jaundice. Most are intrahepatic, and pure extrahepatic biliary cystadenoma is less common. Cases are more common in women. Unless suspected, diagnosis of extrahepatic biliary cystadenoma is often delayed. Here, we report the case of a young woman with extrahepatic biliary cystadenoma who presented at Raja Isteri Pengiran Anak Saleha Hospital with obstructive jaundice initially thought to be due to a large biliary stone based on the endoscopic cholangiogram image. She was successfully managed with resection of the cystadenoma. [ABSTRACT FROM AUTHOR]

Pembrolizumab, an anti-programmed cell death (PD)-1 monoclonal antibody, is an anticancer agent showing substantial benefit in lung cancer and melanoma treatment. Biliary tract cancer (BTC) has been shown to respond to pembrolizumab; however, no credible data of such treatment outcomes exist. Therefore, we assessed the clinical outcomes and safety of pembrolizumab in patients with gemcitabine/cisplatin-refractory BTC. In this multicenter study, we retrospectively analyzed 51 patients with programmed cell death 1-ligand 1 (PD-L1)-positive gemcitabine/cisplatin-refractory BTC treated with pembrolizumab in four tertiary hospitals in Korea. PD-L1 positivity was defined as the expression of PD-L1 in ≥1% of tumor cells based on immunohistochemical staining (22C3, SP263, and E1L3N assays). The median age of the patients was 66 (range, 43–83) years and 29 (56.9%) were male. Extrahepatic cholangiocarcinoma was the most common cancer type (n = 30, 58.8%). Partial response and stable disease were achieved in 5 (9.8%) and 13 (25.5%) patients, respectively. Median progression-free survival and overall survival were 2.1 (95% CI, 1.7–2.4) and 6.9 (95% CI, 5.4–8.3) months, respectively. Overall, 30 (58.8%) patients experienced treatment-related adverse events (AEs). Only four (7.8%) patients experienced grades 3 and 4 AEs. In PD-L1-positive gemcitabine/cisplatin-refractory BTC, pembrolizumab presented durable efficacy, with a 9.8% response rate and manageable toxicity. [ABSTRACT FROM AUTHOR]