Acanthosis nigricans malin révélant un cholangiocarcinome

IntroductionAcanthosis nigricans is a dermatosis characterized by the presence of a hyperpigmented, velvety cutaneous thickening in the flexural areas, especially axillary and inguinal fossas, and lateral faces of the neck. AN is usually a benign condition but can sometimes reveal an internal malignancy corresponds to a cutaneous paraneoplasic syndrome. Literature shows a predominant association with gastric adenocarcinoma. Here, we report a rare association between AN and cholangiocarcinoma.Case reportWe report a 43-year-old woman who presented an extensive AN associated to a tripe palms syndrome and florid cutaneous papillomatosis. She consulted in dermatology because of the itchiness of the lesions as well as for esthetics reasons. Complementary investigations enabled to diagnose a cholangiocarcinoma without visceral metastasis and she was treated by tumor resection and chemotherapy. Consequently, a slight improvement of the skin condition and the disappearance of pruritus were observed.ConclusionAN should be considered as cutaneous sign either of malignancy or endocrinopathy and therefore requires further investigations. The existence of extensive lesions, pruritus, tripe palms syndrome, florid cutaneous papillomatosis or mucous lesions, associated to an AN is a sign of malignancy should be investigated urgently the early diagnosis of which can lead to a better prognosis.     

Cholecystectomy reduces subsequent cholangiocarcinoma risk in choledocholithiasis patients undergoing endoscopic intervention

BACKGROUNDCholangiocarcinoma is a disease with a high mortality rate. Our previous study revealed that cholelithiasis patients who undergo endoscopic sphincterotomy (ES)/endoscopic papillary balloon dilatation are at a higher risk for subsequent cholangiocarcinoma than cholelithiasis patients who undergo cholecystectomy.AIMTo clarify the relationship between recurrent biliary events and subsequent cholangiocarcinoma risk in choledocholithiasis patients.METHODSFrom one million random cases in the Taiwan National Health Insurance Research Database 2004–2011, we selected symptomatic choledocholithiasis patients older than 18 years who were admitted from January 2005 to December 2009 (study group). Cases for a control group were defined as individuals who had never been diagnosed with cholelithiasis, matched by sex and age in a 1:3 ratio. The study group was further divided into ES/endoscopic papillary balloon dilatation, both ES/endoscopic papillary balloon dilatation and cholecystectomy, and no intervention groups.RESULTSWe included 2096 choledocholithiasis patients without previous intervention or cholangiocarcinoma. A total of 12 (2.35%), 11 (0.74%), and 1 (1.00%) subsequent cholangiocarcinoma cases were diagnosed among 511 ES/endoscopic papillary balloon dilatation patients, 1485 patients with no intervention, and 100 ES/endoscopic papillary balloon dilatation and cholecystectomy patients, respectively. The incidence rates of recurrent biliary event were 527.79/1000 person-years and 286.69/1000 person-years in the subsequent cholangiocarcinoma and no cholangiocarcinoma group, showing a high correlation between subsequent cholangiocarcinoma risk and recurrent biliary events.CONCLUSIONCholedocholithiasis patients who undergo further cholecystectomy after ES/endoscopic papillary balloon dilatation have decreased subsequent cholangiocarcinoma risk due to reduced recurrent biliary events.     

Geoepidemiologic variation in outcomes of primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, progressive, hepatobiliary disease characterized by inflammation and fibrosis of the intra- and extra-hepatic bile ducts. Its natural history is one that generally progresses towards cirrhosis, liver failure, cholangiocarcinoma, and ultimately disease-related death, with a median liver transplantation-free survival time of approximately 15-20 years. However, despite its lethal nature, PSC remains a heterogenous disease with significant variability in outcomes amongst different regions of the world. There are also many regions where the outcomes of PSC have not been studied, limiting the overall understanding of this disease worldwide. In this review, we present the geoepidemiologic variations in outcomes of PSC, with a focus on survival pre- and post-liver transplantation as well as the concurrence of inflammatory bowel disease and hepatobiliary neoplasia.     

胆管癌组织的环状RNA circ?ITCH水平及临床意义

目的探讨胆管癌组织的环状RNA circ-ITCH水平及临床意义。方法收集本院2014年1月至2015年12月手术切除的95对胆管癌组织和癌旁组织,采用实时定量PCR(QPCR)检测上述组织的circ-ITCH水平,比较癌组织和癌旁组织的circ-ITCH水平差异,进一步分析circ-ITCH水平与胆管癌临床病理特征和预后的关系。结果胆管癌组织的circ-ITCH水平[中位数(四分位数)]为0.654(0.407~1.219),低于癌旁组织中的2.034(1.079~3.749),差异有统计学意义(P<0.05)。circ-ITCH水平与年龄、性别、吸烟、饮酒、HBV感染、肿瘤位置、分化程度和CEA水平无关(P>0.05),而与ECOG评分、T分期、N分期、TNM分期和CA199水平有关(P<0.05)。circ-ITCH高水平者的中位总生存期为35.0(95%CI:24.6~45.4)个月,长于低水平者的8.0(95%CI:7.3~28.7)个月(P<0.05);多因素分析显示circ-ITCH水平是影响胆管癌预后的独立因素(HR=0.535,95%CI:0.343~0.835,P=0.006)。结论胆管癌组织中circ-ITCH低表达,且与胆管癌患者的ECOG评分和临床分期有关,circ-ITCH低水平者的预后较差,在胆管癌诊断和预后预测上有一定价值。     

Current standards and future perspectives in adjuvant treatment for biliary tract cancers

Biliary tract cancer, including cholangiocarcinoma (CCA) and gallbladder cancer (GBC) are rare tumours with a rising incidence. Prognosis is poor, since most patients are diagnosed with advanced disease. Only ~20% of patients are diagnosed with early-stage disease, suitable for curative surgery. Despite surgery performed with potentially-curative intent, relapse rates are high, with around 60–70% of patients expected to have disease recurrence. Most relapses occur in the form of distant metastases, with a predominance of liver spread. In view of high tumour recurrence, adjuvant strategies have been explored for many years, in the form of radiotherapy, chemo-radiotherapy and chemotherapy. Historically, few randomised trials were available, which included a variety of additional tumours (e.g. pancreatic and ampullary tumours); most evidence relied on phase II and retrospective studies, with no high-quality evidence available to define the real benefit derived from adjuvant strategies.Since 2017, three randomised phase III clinical trials have been reported; all recruited patients with resected biliary tract cancer (CCA and GBC) who were randomised to observation alone, or chemotherapy in the form of gemcitabine (BCAT study; included patients diagnosed with extrahepatic CCA only), gemcitabine and oxaliplatin (PRODIGE-12/ACCORD-18; included patients diagnosed with CCA and GBC) or capecitabine (BILCAP; included patients diagnosed with CCA and GBC). While gemcitabine-based chemotherapy failed to show an impact on patient outcome (relapse-free survival (RFS) or overall survival (OS)), the BILCAP study showed a benefit from adjuvant capecitabine in terms of OS (pre-planned sensitivity analysis in the intention-to-treat population and in the per-protocol analysis), with confirmed benefit in terms of RFS. Based on the BILCAP trial, international guidelines recommend adjuvant capecitabine for a period of six months following potentially curative resection of CCA as the current standard of care for resected CCA and GBC. However, BILCAP failed to show OS benefit in the intention-to-treat (non-sensitivity analysis) population (primary end-point), and this finding, as well as some inconsistencies between studies has been criticised and has led to confusion in the biliary tract cancer medical community.This review summarises the adjuvant field in biliary tract cancer, with evidence before and after 2017, and comparison between the latest randomised phase III studies. Potential explanations are presented for differential findings, and future steps are explored.