BACKGROUND:Stenting of malignant hilar strictures remains a standard endoscopic treatment in patients with unresectable tumors.The aim of this two-center prospective study was to compare unilateral versus bilateral dr...BACKGROUND:Stenting of malignant hilar strictures remains a standard endoscopic treatment in patients with unresectable tumors.The aim of this two-center prospective study was to compare unilateral versus bilateral drainage in hilar malignant stenosis Bismuth-Corlette type II.METHODS:During a 3-year period,a total of 49 patients with hilar tumors(Bismuth-Corlette type II) were referred for endoscopic treatment,following the criteria of unresectability.Ultrasound,computed tomography scan and magnetic resonance cholangiopancreatography(MRCP) were previously performed in all patients in order to facilitate endoscopic retrograde cholangiopancreatography(ERCP).The stricture was first passed by the hydrophilic guide-wire and then contrast medium was injected.Mechanical bile duct dilation was performed,followed by plastic stent placement only in the liver lobe which was previously opacified.The procedures were performed under conscious sedation.The patients were followed up for the next 12 months with a stent exchange every 3 months.Primary outcome was assessed by patient survival in the first 12 months after the procedure.RESULTS:All 49 patients were treated with ERCP while 39(79.59%) had successful stent placement.Among these,32 had hilar cholangiocarcinoma(82%) and 7(18%) had gallbladder cancer.Two groups of patients had Bismuth II strictures:A,21 patients(54%) with unilateral contrast injection and drainage,and B,18(46%) with bilateral contrast injection and drainage.A total of 57 plastic stents were used(10 Fr,89%;11.5 Fr,11%).Group B showed a lower bilirubin level 7 days after the procedure(P=0.008).Early complications were cholangitis(3 patients,2 in group A and 1 in group B) and acute pancreatitis(2 patients,1 each in A and B) with no statistical difference between the groups.Late complications were stent migration(5 patients,1 in A and 4 in B) and stent clogging(6 patients,2 in A and 4 in B) showing a significant difference between the groups(P<0.01).The first stent replacement after 3 months was successful in 展开更多
BACKGROUND:Endoscopic therapy has been successful in the management of biliary complications after both deceased donor liver transplantation(DDLT) and living donor liver transplantation(LDLT).LDLT is thought to be ass...BACKGROUND:Endoscopic therapy has been successful in the management of biliary complications after both deceased donor liver transplantation(DDLT) and living donor liver transplantation(LDLT).LDLT is thought to be associated with higher rates of biliary complications,but there are few studies comparing the success of endoscopic management of anastomotic strictures between the two groups.This study aims to compare our experience in the endoscopic management of anastomotic strictures in DDLT versus LDLT.METHODS:This is a retrospective database review of all liver transplant patients undergoing endoscopic retrograde cholangiopancreatography(ERCP) after liver transplantation.The frequency of anastomotic stricture and the time to develop and to resolve anastomotic stricture were compared between DDLT and LDLT.The response of anastomotic stricture to endoscopic therapy was also analyzed.RESULTS:A total of 362 patients underwent liver transplantation between 2003 and 2011,with 125 requiring ERCP to manage biliary complications.Thirty-three(9.9%) cases of DDLT and 8(27.6%) of LDLT(P=0.01) were found to have anastomotic stricture.When comparing DDLT and LDLT,there was no difference in the mean time to the development of anastomotic strictures(98±17 vs 172±65 days,P=0.11),likelihood of response to ERCP [22(66.7%) vs 6(75.0%),P=0.69],mean time to the resolution of anastomotic strictures(268±77 vs 125±37 days,P=0.34),and the number of ERCPs required to achieve resolution(3.9±0.4 vs 4.7±0.9,P=0.38).CONCLUSIONS:Endoscopic therapy is effective in the majority of biliary complications relating to liver transplantation.Anastomotic strictures occur more frequently in LDLT compared with DDLT,with equivalent endoscopic treatment response and outcomes for both groups.展开更多
BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity an...BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.展开更多
BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts.This study aimed to demonstrate the hepatic and extrahepatic characteris...BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts.This study aimed to demonstrate the hepatic and extrahepatic characteristic findings and prognostic outcomes of Turkish patients with PSC. METHODS:The medical records of 35 consecutive patients with PSC from January 1988 to June 2007 were recorded prospectively.From the time of diagnosis,clinical features and laboratory data were collected. RESULTS:The mean age of the 35 patients was 41.69 years (range 15-80 years)at the time of diagnosis;14(40%)were female,and 21(60%)were male.The mean duration of follow-up was 58.86 months(1-180 months).Twenty(57.1%) of the patients with PSC were asymptomatic and 22(62.9%) had inflammatory bowel disease.At the time of diagnosis, 20(57.1%)of the patients had both intra-and extra-hepatic PSC.Twenty-one(60%)of the patients,who had undergone ERCP for stent placement,had dominant bile duct stenosis. Cholangiocarcinoma was found in 2(5.7%)of the patients and cirrhosis was detected in 7(20%);5(14.3%)underwent liver transplantation.The median follow-up time after liver transplantation was 23 months and all are still alive.Six (17.1%)patients died. CONCLUSIONS:PSC has a clinical course varied from advanced liver disease requiring liver transplantation within a short time to being asymptomatic for decades. The prognosis of Turkish patients with PSC is also disappointing as described in other studies.展开更多
AIM:To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.METHODS:Between January 1992 and June 2012,28 patients affected by duodenopancreatic endocrine tumors ...AIM:To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.METHODS:Between January 1992 and June 2012,28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1(MEN1)syndrome underwent surgery at our institution.This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome(ZES).Surgical treatment consisted of duodenopancreatectomy(DP)or total pancreatectomy(TP).Regional lymphadenectomy was always performed.Any hepatic tumoral lesions found were removed during surgery.In MEN1 patients,removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia.One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors.This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.RESULTS:Seventeen MEN1 patients affected with ZES were analyzed.The mean age was 40 years.Fifteen patients underwent DP and two TP.On histopathological examination,duodeno pancreatic endocrine tumors were found in all 17 patients.Eighty-one gastrinomas were detected in the first three portions of the duodenum.Only one gastrinoma was found in the pancreas.The mean number of gastrinomas per patient was 5(range 1-16).Malignancy was established in 12 patients(70.5%)after lymph node,liver and omental metastases were found.Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s).In two cases,the ectopic gastrinoma was removed at the same time as pancreatic surgery,while in the third case,the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.CONCLUSION:These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.展开更多
AIM:To investigate the roles of peribiliary glands around the bile ducts in the pathophysiology of the biliary tract.METHODS:The expression of fetal pancreatic markers,pancreatic duodenal homeobox factor 1(PDX1)and ha...AIM:To investigate the roles of peribiliary glands around the bile ducts in the pathophysiology of the biliary tract.METHODS:The expression of fetal pancreatic markers,pancreatic duodenal homeobox factor 1(PDX1)and hairy and enhancer of split 1(HES1)and endodermal stem/progenitor(S/P)cell markers[CD44s,chemokine receptor type 4(CXCR4),SOX9 and epithelial cell adhesion molecule(EpCAM)]were examined immunohistochemically in 32 normal adult livers(autopsy livers)and 22 hepatolithiatic livers(surgically resected livers).The latter was characterized by the proliferation of the peribiliary glands.Immunohistochemistry was performed using formalin-fixed,paraffin-embedded tissue sections after deparaffinization.Although PDX1and HES1 were expressed in both the nucleus and cytoplasm of epithelial cells,only nuclear staining was evaluated.SOX9 was expressed in the nucleus,while CD44s,CXCR4 and EpCAM were expressed in the cell membranes.The frequency and extent of the expression of these molecules in the lining epithelia and peribiliary glands were evaluated semi-quantitatively based on the percentage of positive cells:0,1+(focal),2+(moderate)and 3+(extensive).RESULTS:In normal livers,PDX1 was infrequently expressed in the lining epithelia,but was frequently expressed in the peribiliary glands.In contrast,HES1was frequently expressed in the lining epithelia,but its expression in the peribiliary glands was focal,suggesting that the peribiliary glands retain the potential of differentiation toward the pancreas and the lining epithelia exhibit properties to inhibit such differentiation.This unique combination was also seen in hepatolithiatic livers.The expression of endodermal S/P cell markers varied in the peribiliary glands in normal livers:SOX9 and EpCAM were frequently expressed,CD44s infrequently,and CXCR4 almost not at all.The expression of these markers,particularly CD44s and CXCR4,increased in the peribiliary glands and lining epithelia in hepatolithiatic livers.This increased expression of endodermal S/P cell markers ma展开更多
目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关...目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关并发症进行随访。结果6例RMS/BT患儿中男5例、女1例,中位发病年龄28.5个月(7~50个月),中位随访时间21个月(3~43个月)。原发于肝内胆道2例,原发于肝外胆道3例,原发于肝门部胆总管近端和左右肝管1例。肝内型主要以腹痛或局部包块为原发症状就诊,肝外型和肝门部肿瘤主要以胆道梗阻表现为首发症状就诊。根据横纹肌肉瘤治疗前TNM(tumor-node-metastasis)临床分期标准,T 2b N 0M 0期2例,T 2b N xM 0期3例,T 1b N 0M 0期1例;根据美国横纹肌肉瘤研究组术后-病理分期系统,5例为Ⅲ期,1例为Ⅰ期。RMS/BT患儿采取以手术、化疗及放疗为主的综合性治疗模式。2例行一期肿瘤切除术,4例行肿瘤活检化疗后再手术。术后6例均行化疗,3例进行了原发部位的放疗,未结束化疗的1例拟接受放射治疗。在治疗过程中1例发生肿瘤破裂、胆瘘、胆汁性腹膜炎。本组6例RMS/BT患儿中,5例存活,2例复发,1例死亡。存活的5例中,2例结束治疗无瘤生存,2例仍在治疗中,1例肿瘤复发继续治疗中。结论RMS/BT对化疗敏感,遵循肿瘤活检、化疗、延迟手术及放疗的诊疗流程,大多能达到肿瘤根治性切除、有效减少局部复发的目的。展开更多
Recent histological and molecular characterization of cholangiocarcinoma(CCA) highlights the heterogeneity of this cancer that may emerge at different sites of the biliary tree and with different macroscopic or morpho...Recent histological and molecular characterization of cholangiocarcinoma(CCA) highlights the heterogeneity of this cancer that may emerge at different sites of the biliary tree and with different macroscopic or morphological features.Furthermore,different stem cell niches have been recently described in the liver and biliarytree,suggesting this as the basis of the heterogeneity of intrahepatic(IH)-and extrahepatic(EH)-CCAs,which are two largely different tumors from both biological and epidemiological points of view.The complexity of the organization of the liver stem cell compartments could underlie the CCA clinical-pathological heterogeneity and the criticisms in classifying primitive liver tumors.These recent advances highlight a possible new classification of CCAs based on cells of origin and this responds to the need of generating homogenous diagnostic,prognostic and,hopefully,therapeutic categories of IH-and EH-CCAs.展开更多
基金supported by the Ministry of Education and Science of the Republic of Serbia(175054)
文摘BACKGROUND:Stenting of malignant hilar strictures remains a standard endoscopic treatment in patients with unresectable tumors.The aim of this two-center prospective study was to compare unilateral versus bilateral drainage in hilar malignant stenosis Bismuth-Corlette type II.METHODS:During a 3-year period,a total of 49 patients with hilar tumors(Bismuth-Corlette type II) were referred for endoscopic treatment,following the criteria of unresectability.Ultrasound,computed tomography scan and magnetic resonance cholangiopancreatography(MRCP) were previously performed in all patients in order to facilitate endoscopic retrograde cholangiopancreatography(ERCP).The stricture was first passed by the hydrophilic guide-wire and then contrast medium was injected.Mechanical bile duct dilation was performed,followed by plastic stent placement only in the liver lobe which was previously opacified.The procedures were performed under conscious sedation.The patients were followed up for the next 12 months with a stent exchange every 3 months.Primary outcome was assessed by patient survival in the first 12 months after the procedure.RESULTS:All 49 patients were treated with ERCP while 39(79.59%) had successful stent placement.Among these,32 had hilar cholangiocarcinoma(82%) and 7(18%) had gallbladder cancer.Two groups of patients had Bismuth II strictures:A,21 patients(54%) with unilateral contrast injection and drainage,and B,18(46%) with bilateral contrast injection and drainage.A total of 57 plastic stents were used(10 Fr,89%;11.5 Fr,11%).Group B showed a lower bilirubin level 7 days after the procedure(P=0.008).Early complications were cholangitis(3 patients,2 in group A and 1 in group B) and acute pancreatitis(2 patients,1 each in A and B) with no statistical difference between the groups.Late complications were stent migration(5 patients,1 in A and 4 in B) and stent clogging(6 patients,2 in A and 4 in B) showing a significant difference between the groups(P<0.01).The first stent replacement after 3 months was successful in
文摘BACKGROUND:Endoscopic therapy has been successful in the management of biliary complications after both deceased donor liver transplantation(DDLT) and living donor liver transplantation(LDLT).LDLT is thought to be associated with higher rates of biliary complications,but there are few studies comparing the success of endoscopic management of anastomotic strictures between the two groups.This study aims to compare our experience in the endoscopic management of anastomotic strictures in DDLT versus LDLT.METHODS:This is a retrospective database review of all liver transplant patients undergoing endoscopic retrograde cholangiopancreatography(ERCP) after liver transplantation.The frequency of anastomotic stricture and the time to develop and to resolve anastomotic stricture were compared between DDLT and LDLT.The response of anastomotic stricture to endoscopic therapy was also analyzed.RESULTS:A total of 362 patients underwent liver transplantation between 2003 and 2011,with 125 requiring ERCP to manage biliary complications.Thirty-three(9.9%) cases of DDLT and 8(27.6%) of LDLT(P=0.01) were found to have anastomotic stricture.When comparing DDLT and LDLT,there was no difference in the mean time to the development of anastomotic strictures(98±17 vs 172±65 days,P=0.11),likelihood of response to ERCP [22(66.7%) vs 6(75.0%),P=0.69],mean time to the resolution of anastomotic strictures(268±77 vs 125±37 days,P=0.34),and the number of ERCPs required to achieve resolution(3.9±0.4 vs 4.7±0.9,P=0.38).CONCLUSIONS:Endoscopic therapy is effective in the majority of biliary complications relating to liver transplantation.Anastomotic strictures occur more frequently in LDLT compared with DDLT,with equivalent endoscopic treatment response and outcomes for both groups.
文摘BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.
文摘BACKGROUND:Primary sclerosing cholangitis(PSC)is a chronic cholestatic liver disease characterized by destruction and fibrosis of the bile ducts.This study aimed to demonstrate the hepatic and extrahepatic characteristic findings and prognostic outcomes of Turkish patients with PSC. METHODS:The medical records of 35 consecutive patients with PSC from January 1988 to June 2007 were recorded prospectively.From the time of diagnosis,clinical features and laboratory data were collected. RESULTS:The mean age of the 35 patients was 41.69 years (range 15-80 years)at the time of diagnosis;14(40%)were female,and 21(60%)were male.The mean duration of follow-up was 58.86 months(1-180 months).Twenty(57.1%) of the patients with PSC were asymptomatic and 22(62.9%) had inflammatory bowel disease.At the time of diagnosis, 20(57.1%)of the patients had both intra-and extra-hepatic PSC.Twenty-one(60%)of the patients,who had undergone ERCP for stent placement,had dominant bile duct stenosis. Cholangiocarcinoma was found in 2(5.7%)of the patients and cirrhosis was detected in 7(20%);5(14.3%)underwent liver transplantation.The median follow-up time after liver transplantation was 23 months and all are still alive.Six (17.1%)patients died. CONCLUSIONS:PSC has a clinical course varied from advanced liver disease requiring liver transplantation within a short time to being asymptomatic for decades. The prognosis of Turkish patients with PSC is also disappointing as described in other studies.
文摘AIM:To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.METHODS:Between January 1992 and June 2012,28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1(MEN1)syndrome underwent surgery at our institution.This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome(ZES).Surgical treatment consisted of duodenopancreatectomy(DP)or total pancreatectomy(TP).Regional lymphadenectomy was always performed.Any hepatic tumoral lesions found were removed during surgery.In MEN1 patients,removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia.One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors.This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.RESULTS:Seventeen MEN1 patients affected with ZES were analyzed.The mean age was 40 years.Fifteen patients underwent DP and two TP.On histopathological examination,duodeno pancreatic endocrine tumors were found in all 17 patients.Eighty-one gastrinomas were detected in the first three portions of the duodenum.Only one gastrinoma was found in the pancreas.The mean number of gastrinomas per patient was 5(range 1-16).Malignancy was established in 12 patients(70.5%)after lymph node,liver and omental metastases were found.Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s).In two cases,the ectopic gastrinoma was removed at the same time as pancreatic surgery,while in the third case,the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.CONCLUSION:These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.
文摘AIM:To investigate the roles of peribiliary glands around the bile ducts in the pathophysiology of the biliary tract.METHODS:The expression of fetal pancreatic markers,pancreatic duodenal homeobox factor 1(PDX1)and hairy and enhancer of split 1(HES1)and endodermal stem/progenitor(S/P)cell markers[CD44s,chemokine receptor type 4(CXCR4),SOX9 and epithelial cell adhesion molecule(EpCAM)]were examined immunohistochemically in 32 normal adult livers(autopsy livers)and 22 hepatolithiatic livers(surgically resected livers).The latter was characterized by the proliferation of the peribiliary glands.Immunohistochemistry was performed using formalin-fixed,paraffin-embedded tissue sections after deparaffinization.Although PDX1and HES1 were expressed in both the nucleus and cytoplasm of epithelial cells,only nuclear staining was evaluated.SOX9 was expressed in the nucleus,while CD44s,CXCR4 and EpCAM were expressed in the cell membranes.The frequency and extent of the expression of these molecules in the lining epithelia and peribiliary glands were evaluated semi-quantitatively based on the percentage of positive cells:0,1+(focal),2+(moderate)and 3+(extensive).RESULTS:In normal livers,PDX1 was infrequently expressed in the lining epithelia,but was frequently expressed in the peribiliary glands.In contrast,HES1was frequently expressed in the lining epithelia,but its expression in the peribiliary glands was focal,suggesting that the peribiliary glands retain the potential of differentiation toward the pancreas and the lining epithelia exhibit properties to inhibit such differentiation.This unique combination was also seen in hepatolithiatic livers.The expression of endodermal S/P cell markers varied in the peribiliary glands in normal livers:SOX9 and EpCAM were frequently expressed,CD44s infrequently,and CXCR4 almost not at all.The expression of these markers,particularly CD44s and CXCR4,increased in the peribiliary glands and lining epithelia in hepatolithiatic livers.This increased expression of endodermal S/P cell markers ma
文摘目的分析儿童胆道横纹肌肉瘤(rhabdomyosarcoma of the biliary tree,RMS/BT)的临床特点、治疗策略及预后情况。方法回顾性研究2013年1月至2020年3月首都医科大学附属北京儿童医院诊治的6例RMS/BT患儿临床资料,并对患儿预后情况及相关并发症进行随访。结果6例RMS/BT患儿中男5例、女1例,中位发病年龄28.5个月(7~50个月),中位随访时间21个月(3~43个月)。原发于肝内胆道2例,原发于肝外胆道3例,原发于肝门部胆总管近端和左右肝管1例。肝内型主要以腹痛或局部包块为原发症状就诊,肝外型和肝门部肿瘤主要以胆道梗阻表现为首发症状就诊。根据横纹肌肉瘤治疗前TNM(tumor-node-metastasis)临床分期标准,T 2b N 0M 0期2例,T 2b N xM 0期3例,T 1b N 0M 0期1例;根据美国横纹肌肉瘤研究组术后-病理分期系统,5例为Ⅲ期,1例为Ⅰ期。RMS/BT患儿采取以手术、化疗及放疗为主的综合性治疗模式。2例行一期肿瘤切除术,4例行肿瘤活检化疗后再手术。术后6例均行化疗,3例进行了原发部位的放疗,未结束化疗的1例拟接受放射治疗。在治疗过程中1例发生肿瘤破裂、胆瘘、胆汁性腹膜炎。本组6例RMS/BT患儿中,5例存活,2例复发,1例死亡。存活的5例中,2例结束治疗无瘤生存,2例仍在治疗中,1例肿瘤复发继续治疗中。结论RMS/BT对化疗敏感,遵循肿瘤活检、化疗、延迟手术及放疗的诊疗流程,大多能达到肿瘤根治性切除、有效减少局部复发的目的。
基金Supported by Research Project Grant from the University "Sapienza" of RomeFIRB grant No. RBAP10Z7FS_001+3 种基金FIRB grant No. RBAP10Z7FS_004PRIN grant No. 2009X84L84_001 (to Gaudio E)PRIN grant No. 2009X84L84_002 (to Alvaro D)Consorzio Interuniversitario Trapianti d'Organo,Rome,Italy
文摘Recent histological and molecular characterization of cholangiocarcinoma(CCA) highlights the heterogeneity of this cancer that may emerge at different sites of the biliary tree and with different macroscopic or morphological features.Furthermore,different stem cell niches have been recently described in the liver and biliarytree,suggesting this as the basis of the heterogeneity of intrahepatic(IH)-and extrahepatic(EH)-CCAs,which are two largely different tumors from both biological and epidemiological points of view.The complexity of the organization of the liver stem cell compartments could underlie the CCA clinical-pathological heterogeneity and the criticisms in classifying primitive liver tumors.These recent advances highlight a possible new classification of CCAs based on cells of origin and this responds to the need of generating homogenous diagnostic,prognostic and,hopefully,therapeutic categories of IH-and EH-CCAs.
