Hemofilia paraneoplásica adquirida secundaria a adenocarcinoma de la ampolla de Vater / Acquired paraneoplastic hemophilia secondary to Vater's ampulla adenocarcinoma

Resumen La hemofilia adquirida A es un desorden hemorrágico inusual de origen autoinmune que resulta en la formación de autoanticuerpos dirigidos contra el factor VIII de la coagulación. Estos autoanticuer pos pueden actuar neutralizando parcial o completamente la activación o función del factor, o también pueden acelerar su eliminación de la circulación. La incidencia mundial de la enfermedad es de 1.5 casos por millón de habitantes por año. En cerca del 50% de los pacientes se puede detectar una enfermedad subyacente que se presume responsable de la producción de los autoanticuerpos. Se presenta el caso de un varón con hemofilia adquirida A, en contexto de adenocarcinoma de la ampolla de Vater.

Abstract Acquired hemophilia A is an unusual bleeding disorder of autoimmune origin resulting in the formation of autoantibodies directed against coagulation factor VIII. These autoantibodies can act by partially or completely neutralizing the activation or function of the factor, or they can also accelerate its elimination from the circulation. The global incidence of the disease is 1.5 cases per million inhabitants per year. In nearly 50% of cases, an underlying disease that is presumed responsible to produce autoantibodies can be detected. We report a case with acquired hemophilia A, in a patient with Vater's ampulla adenocarcinoma.

Clinical features and prognosis of extrahepatic biliary neuroendocrine neoplasms / 中华普通外科杂志

Objective@#To investigate the clinical features and prognostic factors of extrahepatic biliary neuroendocrine neoplasms(EB-NENs).@*Methods@#The clinical data and survival of 21 patients with EB-NENs admitted from May 2014 to May 2018 were analyzed retrospectively.@*Results@#These 21 EB-NENs patients accounted for 1.6% (21/1313) of all biliary tract neoplasms treated during the study period. Seven (33.3%) cases had lymph node metastasis. Five (23.8%) suffered from distant metastasis. The follow up time was (4~46.5) months with median survival time of (23.23 ±4.17) months. Univariate analysis showed that the risk factors for survival were tumor TNM stage (χ2=9.066, P=0.003), lymph node metastasis(χ2=6.399, P=0.011) and distant metastasis (χ2=9.808, P=0.002). By multivariate analysis, independent risk factors were tumor TNM stage(P=0.008, RR=3.003, 95% CI: 1.332~6.774), lymph node metastasis(P=0.023, RR=5.382, 95% CI: 1.261~22.971) and distant metastasis or not(P=0.007, RR=7.423, 95% CI: 1.730~31.851).@*Conclusion@#EB-NENs was a rare malignant tumor with poor prognosis. Tumor TNM stage, lymph node metastasis and distant metastasis were independent prognostic factors for EB-NENs patients.

Clinical features and prognosis of extrahepatic biliary neuroendocrine neoplasms / 中华普通外科杂志

Objective To investigate the clinical features and prognostic factors of extrahepatic biliary neuroendocrine neoplasms(EB-NENs).Methods The clinical data and survival of 21 patients with EB-NENs admitted from May 2014 to May 2018 were analyzed retrospectively.Results These 21 EB-NENs patients accounted for 1.6％ (21/1313) of all biliary tract neoplasms treated during the study period.Seven (33.3％) cases had lymph node metastasis.Five (23.8％) suffered from distant metastasis.The follow up time was (4 ～ 46.5) months with median survival time of (23.23 ± 4.17) months.Univariate analysis showed that the risk factors for survival were tumor TNM stage (x2 =9.066,P =0.003),lymph node metastasis(x2 =6.399,P=0.011) and distant metastasis (x2 =9.808,P=0.002).By multivariate analysis,independent risk factors were tumor TNM stage (P =0.008,RR =3.003,95％ CI:1.332 ～ 6.774),lyraph node metastasis (P =0.023,RR =5.382,95 ％ CI:1.261 ～ 22.971) and distant metastasis or not(P =0.007,RR =7.423,95％ CI:1.730 ～ 31.851).Conclusion EB-NENs was a rare malignant tumor with poor prognosis.Tumor TNM stage,lymph node metastasis and distant metastasis were independent prognostic factors for EB-NENs patients.

Bile duct segmental resection versus pancreatoduodenectomy for middle and distal common bile duct cancer

PURPOSE: To compare survival outcomes between bile duct segmental resection (BDR) and pancreatoduodenectomy (PD) for the treatment of middle and distal bile duct cancer. METHODS: From 1997 to 2013, a total of 96 patients who underwent curative intent surgery for middle and distal bile duct cancer were identified. The patients were divided into 2 groups based on the type of operation; 20 patients were included in the BDR group and 76 patients were in the PD group. We retrospectively reviewed the clinical outcomes. RESULTS: The number of lymph nodes (LNs) was significantly greater in patients within the PD group compared to the BDR group. The total number of LNs was 6.5 ± 8.2 vs. 11.2 ± 8.2 (P = 0.017) and the number of metastatic LNs was 0.4 ± 0.9 vs. 1.0 ± 1.5 (P = 0.021), respectively. After a median follow-up period of 24 months (range, 4–169 months), the recurrence-free survival of the PD group was superior to that of the BDR group (P = 0.035). In the patients with LN metastases, the patients undergoing PD had significantly better survival than the BDR group (P < 0.001). CONCLUSION: Surgeons should be cautious in deciding to perform BDR for middle and distal common bile duct cancer. PD is recommended if LN metastases are suspected.

A Double-Layered Covered Biliary Metal Stent for the Management of Unresectable Malignant Biliary Obstruction: A Multicenter Feasibility Study

BACKGROUND/AIMS: The covered self-expandable metal stent (CMS) was developed to prevent tumor ingrowth-induced stent occlusion during the treatment of malignant biliary obstruction. However, complications such as cholecystitis, pancreatitis, and stent migration can occur after the endoscopic insertion of CMSs. The aim of the present study was to assess the efficacy and safety of a double-layered CMS (DCMS) for the management of malignant bile duct obstruction. METHODS: DCMSs were endoscopically introduced into 59 patients with unresectable malignant extrahepatic biliary obstruction at four tertiary referral centers, and the patient medical records were retrospectively reviewed. RESULTS: Both the technical and functional success rates were 100%. Procedure-related complications including pancreatitis, cholangitis, stent migration, and liver abscess occurred in five patients (8.5%). The median follow-up period was 265 days (range, 31 to 752 days). Cumulative stent patency rates were 68.2% and 40.8% at 6 and 12 months, respectively. At the final follow-up, the rate of stent occlusion was 33.9% (20/59), and the median stent patency period was 276 days (range, 2 to 706 days). CONCLUSIONS: The clinical outcomes of DCMSs were comparable to the outcomes previously reported for CMSs with respect to stent patency period and complication rates.

A Case of Adenomyomatous Hyperplasia of the Distal Common Bile Duct Mimicking Malignant Stricture / 대한소화기학회지

Adenomyomatous hyperplasia is a reactive malformation or non-neoplastic tumor-like lesion frequently observed in the gallbladder, stomach, duodenum and jejunum, but rare in the extrahepatic bile duct. A 42-year-old man with epigastric discomfort had a stricture in the common bile duct on initial CT scans. Initially, it was regarded as a malignant lesion with some evidence, but histopathologic examinations of multiple biopsies obtained by multiple sessions of endoscopic retrograde cholangiopancreatography showed no evidence of malignancy. The patient had undergone the pylorus preserving pancreaticoduodenectomy because of the possibility of malignancy; however, the final diagnosis was adenomyomatous hyperplasia. It is important to distinguish a malignancy from benign biliary stricture with endoscopic biopsies. Surgery for suspected biliary malignancy often reveals benign lesions. Therefore, a correct diagnosis is important before deciding upon treatment of bile duct stricture. In conclusion, in younger patients with bile duct stricture where there is no evidence of histologic malignancy despite multiple biopsies, the possibility of benign disease such as adenomyomatous hyperplasia should be considered, to avoid unnecessary radical surgery.

Metastatic mucinous adenocarcinoma of the distal common bile duct, from transverse colon cancer presenting as obstructive jaundice

The patient was a 70-year-old male whose chief complaints were obstructive jaundice and weight loss. Abdominal imaging studies showed a 2.5 cm sized mass at the distal common bile duct, which was suggestive of bile duct cancer. Eccentric enhancing wall thickening in the transverse colon was also shown, suggesting concomitant colon cancer. A colonoscopy revealed a lumen-encircling ulcerofungating mass in the transverse colon, that was pathologically proven to be adenocarcinoma. The bile duct pathology was also adenocarcinoma. Pylorus-preserving pancreaticoduodenectomy and extended right hemicolectomy were performed under the diagnosis of double primary cancers. Postoperative histopathologic examination revealed moderately differentiated mucinous adenocarcinoma of transverse colon cancer, and mucinous adenocarcinoma of the distal common bile duct. Immunohistochemical staining studies showed that the bile duct cancer had metastasized from the colon cancer. The patient recovered uneventfully from surgery and will be undergoing chemotherapy for three months.

A Unique Use of a Double-Pigtail Plastic Stent: Correction of Kinking of the Common Bile Duct Due to a Metal Stent

A 72-year-old man with jaundice by ampullary adenocarcinoma was treated at our hospital. For biliary decompression, a transpapillary, fully covered, self-expandable metal stent (FCSEMS) was deployed. Four days later, the patient developed acute cholangitis. Endoscopic carbon dioxide cholangiography revealed kinking of the common bile duct above the proximal end of the FCSEMS. A 7-F double-pigtail plastic stent was therefore placed through the FCSEMS to correct the kink, straightening the common bile duct (CBD) and improving cholangitis. This is the first report of a unique use of a double-pigtail plastic stent to correct CBD kinking. The placement of a double-pigtail plastic stent can correct CBD kinking, without requiring replacement or addition of a FCSEMS, and can lead to cost savings.

Metastatic Common Bile Duct Cancer from Pulmonary Adenocarcinoma Presenting as Obstructive Jaundice / 대한소화기학회지

We report an extremely rare case of metastatic common bile duct cancer from pulmonary adenocarcinoma presenting as obstructive jaundice. The patient was a 76-year-old male, who presented with generalized weakness and right upper quadrant pain. Plain chest X-ray noted multiple small nodules in both lung fields. Abdominal computed tomography scan showed a stricture of the mid common bile duct along with ductal wall enhancement. Endoscopic retrograde cholangiography revealed a concentric, abrupt narrowing of the mid-common bile duct suggestive of primary bile duct cancer. However, pathology comfirmed metastatic common bile duct cancer arising from pulmonary adenocarcinoma with immunohistochemical study with thyroid transcriptional factor-1 (TTF-1).

Clinical efficacy of the technique of reticular basket through cystic duct stone removal during laparoscopic common bile duct exploration / 中华肝胆外科杂志

ObjectiveTo explore the efficacy of the technique of reticular basket through cystic duct stone removal during laparoscopic common bile duct exploration.MethodsFrom January 2008 to February 2011,185 patients received laparoscopic bile duct exploration in our hospital.These patients underwent intraoperative cholangiography (IOC) through the cystic duct with a reticular basket.With the help of a choledochoscope,a reticular basket was used to take out stones for patients who were shadow-positive.Re-cholangiography was then performed to ascertain that no stone was retained in the biliary tract.ResultsOf the 185 patients,60 were found to be positive on IOC.Stones were successfully removed by using a reticular basket through the cystic duct in 45 patients,and through the cystic duct and common bile duct after a micro-incision was made under choledochoscopic assistance in 10 patients.After laparoscopic common bile duct exploration re-cholangiography found no residual stones in these patients. In 5 patients,stones were removed after conversion to open surgery. Conclusions During laparoscopic common bile duct exploration,using reticular baskets via the cystic duct for intraoperative cholangiography (IOC) and removing stones is of great value.

Expression and significance of Survivin and PTEN in human ampullary carcinoma / 中国医师杂志

Objective To study the expressions of survivin and PTEN in ampullary carcinoma and their clinical significance.Methods The expressions of Survivin and PTEN proteins were tested by EnVision immunohistochemistry in 40 cases of ampullary carcinomas and 8 cases of normal ampulla of vater as control.Results The positive rate of Survivin in ampullary carcinomas was significantly higher than normal human controls (82.5％ vs 0％,P ＜ 0.01 ).The differences of the expression of Survivin in ampullaty carcinoma of duodenal invasion,pancreatic invasion,and lymph node metastasis were significant ( P ＜0.05 ).Significant differences were also observed in the expression rate of PTEN hetween the patients with ampullary carcinoma and the normal controls (50％ vs 100％,P ＜ 0.01 ).The differences of the expression of PTEN in ampullary carcinoma of duodenal invasion,pancreatic invasion,and lymph node metastasis were significant ( P ＜ 0.05 ).Significantly negative correlation was found between the expression of Survivin and PTEN by using spearman correlation analysis ( r =-0.57,P ＜ 0.01 ).Conclusions The abnormal expression of Survivin and PTEN gene may promote tumor genesis and progression of ampullary carcinomas and it may be used as the marker for prognosis.

Surgical treatment of hilar cholangiocarcinoma / 中华普通外科杂志

Objective To investigate the clinical features and the prognosis after surgical treatment for hilar cholangiocarcinoma (HCC).Methods The surgical therapy and follow-up result were retrospectively analyzed on 98 cases of hilar cholangiocarcinoma admitted into our hospital from January 1995 to January 2005.Differences between groups were evaluated using Chi-square analysis or Student t-test according to the data type.Survival rate was calculated with Kaplan-Meier method,and using the log-rank test.Results Among 98 patients,83 patients underwent surgical treatment (radical resection in 33,palliative resection in 16,and nonresectional internal or external bile duct drainage in 34),15 patients underwent conservative therapy.The 1-,3-,5-year survival rates were 79％,42％,and 17％ in the resection group and 88％,54％,and 24％ in the radical resection group,respectively.The 1-,3-year survival rates were 55％,and 9％ in palliative resection group,respectively,and none of the patient survived for over 5 years.There were significant differences in the survival rate among the radical resection group and the palliative resection group (log-rank test,P ＜ 0.001).Conclusions Radical resection improves the prognosis of hilar cholangiocarcinoma.

Treatments of choledochal cysts in adults: a study in 169 consecutive patients / 中华肝胆外科杂志

Objective To characterize the spectrum of adult choledochal cyst and to determine suitable surgical procedures for this disorder.Methods The medical records of 169 patients who were treated for choledochal cyst from July 1977 to October 2008 in our Department were retrospectively reviewed.The cysts were classified using Todani's classification as type Ⅰ in 110 patients (65.1％),type Ⅱ in one patient (0.6％),and type Ⅳ in 56 patients (33.1％).The choledochal cysts in two patients could not be classified because of the lack of clinical data.163 patients received surgical treatment,which included cystectomy (n=119),cystenterosotmy (n=33),T-tube drainage following bile duct exploration (n=5),surgical exploration/biopsy (n =5),and endoscopic sphincterotomy (n =1).Results There was an increase in the number of patients in the 1980s which became stable at round 30 patients per every four years since the late 1990s.The proportion of patients presenting with a history of biliary surgery decreased and accounted for about 25％ of patients in the past decades.The rate of cyst malignancy dropped from 16.5％ in 1977=1995 to 9.7％ in 1996=2008.Of the 163 patients who received surgical treatment,long-term complications included anastomotic strictures and intrahepatic bile ductal stones presenting with repeated cholangitis.Conclusions The incidence of adult congenital choledochal cysts had become stable following years of rapid increase.The rate of cancer progression had gradually decreased.The use of different surgical treatments based on the condition of the proximal bile duct helped to prevent postoperative stenosis of biliary anastomosis.

Resultados actuales de la pancreatoduodenectomía para el tratamiento de los tumores periampulares y análisis de factores pronósticos de sobrevida / Results of pancreaticoduodenectomy in the treatment of periampullary tumors

Background: The diagnosis and treatment of periampullary tumors represents a challenge for current medicine. Aim: To review the results of pancreaticoduodenectomy (PDD) in the treatment of periampullary tumors and to identify risk factors that impact the long-term survival. Patients and Methods: We performed a retrospective study of patients who underwent a PDD for periampullary tumors between 1993 and 2009. We reviewed perioperative results and long term survival. We performed a multivariate analysis for long-term survival. Results: A PDD was performed in 181 patients aged 58 ± 12 years (98 females). Piloric preservation was done in 53 percent and a pancreatogastric anastomosis was used in 94 percent of cases. Morbidity was 62 percent and postoperative mortality was 5.5 percent. Pancreatic cancer was the most frequent pathological finding in 41 percent, followed by ampullary cancer in 28 percent and distal bile duct cancer in 16 percent. Median survival was 17 months, with a five years survival of 24 percent. Survival for ampullary tumors was 28 months with a five years survival of 32 percent. The median and five years survival were 14 months and 16 percent for bile duct cancer and 11 months and 14 percent for pancreatic cancer. Multivariate analysis identified tumor type (pancreas /bile duct) and lymph node dissemination as independent predictors of mortality. Conclusions: One quarter of patients experienced long term survival. Mortality predictors were tumor type and lymph node dissemination.

A case of adenomyoma in distal common bile duct / 대한내과학회지

Adenomyoma is a nonneoplastic lesion that can be found anywhere in the gastrointestinal tract, but it's rarely found in the extrahepatic bile duct. To the best of our knowledge, it is a completely benign lesion, but making a clear distinction from malignancy on preoperative evaluation is very difficult. Its clinical importance mainly lies in the possibility that they may be confused with carcinoma, leading to unnecessarily extensive surgical resections. We report here on a case of distal common bile duct adenomyoma that presented with right upper quadrant abdominal pain, and the preoperative examinations could not reveal whether the tumor was benign or malignant. It was finally diagnosed by histological examination after performing pylorus preserving pancreaticoduodenectomy.

Ampulla of Vater Cancer in a Patient with Double Common Bile Duct

Of the various anomalies of the biliary system, a double common bile duct (DCBD) is extremely rare. According to a report by Boyden, the first case of DCBD was reported by Vesalius in 1543, with few additional case reports since 1986. Cancer and Anomalous pancreatobiliary ductal union (APBDU) are the two of the most serious concomitant conditions. A 69-year-old female was admitted to our hospital with the complaint of jaundice. An abdominal computed tomography and percutaneous transhepatic cholangiography revealed a mass lesion at the distal common bile duct. The patient underwent Whipple's operation, and the operative finding showed a septum in the common bile duct, with a DCBD opening as a single duct into the ampulla of Vater. A histological examination showed an adenocarcinoma in the ampulla of Vater. Herein, a case of the ampulla of Vater cancer in a patient with a double common bile duct is reported, with a review of the literature.