Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular disorder characterized by multiple venous malformations involving many organs. BRBNS can occur in various organs, but the most frequently involved organs are the skin and gastrointestinal (GI) tract. GI lesions of BRBNS can cause acute or chronic bleeding, and treatment is challenging. Herein, we report a case of GI BRBNS that was successfully treated with a combination of intraoperative endoscopy and radical resection.
Intestines; Blue rubber bleb nevus syndrome; Bleeding; Hemorrhage
AIM: To investigate the risk factors affecting the liver metastasis (LM) of pancreatic ductal adenocarcinoma (PDAC) after resection.
METHODS: We retrospectively analyzed 101 PDAC patients who underwent surgical resection at the Samsung Medical Center between January 2000 and December 2004. Forty one patients with LM were analyzed for the time of metastasis, prognostic factors affecting LM, and survival.
RESULTS: LM was found in 40.6%. The median time of the LM (n = 41) was 6.0 ± 4.6 mo and most LM occurred within 1 year. In univariate analysis, tumor size, preoperative carbohydrate antigen 19-9, and perineural invasion were factors affecting LM after resection. In multivariate analysis, tumor size was the most important factor for LM. In univariate analysis, tumor cell differentiation was significant to LM in low-risk groups.
CONCLUSION: LM after resection of PDAC occurs early and shows poor survival. Tumor size is the key indicator for LM after resection.
Pancreatic ductal adenocarcinoma; Liver metastasis; Recurrence
Some patients who undergo surgical resection of pancreatic cancer survive longer than other patients. The purpose of this study was to identify the factors that affect long-term survival after resection of histopathologically confirmed pancreatic ductal adenocarcinoma.
A single-center, retrospective study was conducted among 164 patients who underwent surgical resection of pancreatic cancer, between May 1995 and December 2004. The patient follow-up process was conducted via telephone survey and review of electronic medical records for at least 5 years or until death.
We compared patients with long-term (≥60 months, n = 19) and short-term survival (<60 months, n = 145). Resection margin status, differentiation of the tumor, tumor stage, pre-operative serum level of albumin, total bilirubin and carbohydrate antigen (CA) 19-9 level are related with survival difference (all factors, P < 0.05). Multivariate analysis revealed that a pre-operative serum total bilirubin level <7 mg/dL and a pre-operative serum CA19-9 level <37 U/mL is a statistically significant prognostic factor for long-term survival.
The preoperative serum total bilirubin and serum CA19-9 levels are associated with long-term survival after surgical resection of pancreatic cancer.
Pancreatic neoplasms; Bilirubin; CA19-9 antigen
Attempt to identify the beneficial effects associated with surgical procedures on survival outcome of patients with recurrent cholangiocarcinoma.
921 patients diagnosed with cholangiocarcinoma underwent surgical resection with curative intent in a single institute during the last 15 years. Patients with recurrent disease were divided into two groups according to whether surgical procedures were performed for the treatment of recurrence. Clinicopathologic variables, ranges of survival based on sites of recurrence, and types of treatment were analyzed retrospectively.
The median follow-up period was 21.8 months and 316 (34.3%) patients had recurrence. 27 (group A) patients with recurrent disease were treated surgically and 289 patients (group B) were not treated. Liver resection, metastasectomy, pancreaticoduodenectomy, partial pancreatectomy, and regional lymph node dissection were performed on the patients in group A. The overall survival rate was statistically higher in group A (P = 0.001). Among the surgical procedures, resection of locoregional recurrences (except liver) in abdominal cavity (4.0 to 101.8 months vs. 0.6 to 71.6 months) and metastasectomy of abdominal or chest wall (3.5 to 18.9 months vs. 1.9 to 2.2 months) showed remarkable differences with respect to the range of survival.
Better survival outcomes can be expected by performing surgical resection of locoregional recurrences (except liver) in abdominal cavity and abdominal or chest wall metastatic lesions in recurrent cholangiocarcinoma.
Recurrence; Range of survival; Cholangiocarcinoma; Radiofrequency ablation
AIM: To identify risk factors for nonalcoholic steatohepatitis following pancreaticoduodenectomy, with a focus on factors related to pancreatic secretions.
METHODS: The medical records of 228 patients who had a pancreaticoduodenectomy over a 16-mo period were reviewed retrospectively. The 193 patients who did not have fatty liver disease preoperatively were included in the final analysis. Hepatic steatosis was diagnosed using the differences between splenic and hepatic attenuation and liver-to-spleen attenuation as measured by non-enhanced computed tomography.
RESULTS: Fifteen patients (7.8%) who showed postoperative hepatic fatty changes were assigned to Group A, and the remaining patients were assigned to Group B. Patient demographics, preoperative laboratory findings (including levels of C-peptide, glucagon, insulin and glucose tolerance test results), operation types, and final pathological findings did not differ significantly between the two groups; however, the frequency of pancreatic fistula (P = 0.020) and the method of pancreatic duct stenting (P = 0.005) showed significant differences between the groups. A multivariate analysis identified pancreatic fistula (HR = 3.332, P = 0.037) and external pancreatic duct stenting (HR = 4.530, P = 0.017) as independent risk factors for the development of postoperative steatohepatitis.
CONCLUSION: Pancreatic fistula and external pancreatic duct stenting were identified as independent risk factors for the development of steatohepatitis following pancreaticoduodenectomy.
Nonalcoholic fatty liver diseases; Nonalco-holic steatohepatitis; Pancreatic duct stenting; Pancreatic fistula; Pancreatic surgery
AIM: To identify preoperative predictive factors associated with malignancy of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas.
METHODS: Between April 1995 and April 2010, 129 patients underwent surgical resection for IPMNs at our institute and had confirmed pathologic diagnoses. The medical records were retrospectively reviewed and immunohistochemical staining for mucin (MUC) in pancreatic tissues was performed.
RESULTS: Univariate analysis showed that the following five variables were closely associated with malignant IPMNs preoperatively: absence of extrapancreatic malignancy; symptoms; tumor size > 4 cm; main pancreatic duct (MPD) size > 7 mm; and lymph node enlargement on preoperative computed tomography (CT). Multivariate analysis revealed that the following two factors were significantly associated with malignant IPMNs preoperatively: MPD size > 7 mm [odds ratio (OR) = 2.50]; and lymph node enlargement on preoperative CT (OR = 3.57). No significant differences in the expression of MUC1, MUC2 and MUC5AC were observed between benign and malignant IPMNs.
CONCLUSION: MPD size > 7 mm and preoperative lymph node enlargement on CT are useful predictive factors associated with malignancy of IPMNs.
Intraductal papillary mucinous neoplasms; Malignancy; Predictive factors; Pancreatic neoplasms
AIM: To review the clinical course and the management of pseudoaneurysms post-pancreaticoduodenectomy.
METHODS: Medical records of 907 patients who underwent pancreaticoduodenectomies from January 1995 to May 2007 were evaluated retrospectively. The clinical course, management strategy, and outcome of ruptured pseudoaneurysms cases were analyzed.
RESULTS: Twenty-seven (3.0%) of 907 cases had post-operative hemorrhage from ruptured pseudoaneurysms. Pancreatic fistula was evident in 12 (44%) cases. Sentinel bleeding appeared in 21 (77.8%) cases. Of the 27 cases, 11 (41%) cases demonstrated bleeding pseudoaneurysm of the ligated gastroduodenal artery, 8 (30%) of the right, proper, common hepatic artery, 2 (7%) of the right gastric artery, and 4 (15%) of the peripancreatic arteries. The remaining two patients died due to sudden-onset massive hemorrhage and pseudoaneurysm rupture was suspected. Emergent operation was performed on 2 cases directly without angiography. Angiography was attempted in 23 cases. Eighteen (78.2%) cases succeeded to hemostasis; the five failed cases were explored. After embolization of the hepatic artery, five cases developed liver abscesses or infarction and a single case of hepatic failure expired. Gastroduodenal artery embolization with common hepatic artery stent insertion was performed to enhance hepatic artery flow in a single case and was successfully controlled.
CONCLUSION: Bleeding pseudoaneurysms are among the most serious and fatal complications following pancreaticoduodenectomy. Diagnostic angiography has been preferred over endoscopy and is rapidly becoming the standard therapeutic treatment for bleeding pseudoaneurysms.
Pseudoaneurysm; Pseudoaneurysm; Angiography; Embolization; Stent graft
AIM: To compare survival between bile duct segmental resection (BDSR) and pancreaticoduodenectomy (PD) for treating distal bile duct cancers.
METHODS: Retrospective analysis was conducted for 45 patients in a BDSR group and for 149 patients in a PD group.
RESULTS: The T-stage (P < 0.001), lymph node invasion (P = 0.010) and tumor differentiation (P = 0.005) were significant prognostic factors in the BDSR group. The 3- and 5-year overall survival rates for the BDSR group and PD group were 51.7% and 36.6%, respectively and 46.0% and 38.1%, respectively (P = 0.099). The BDSR group and PD group did not show any significant difference in survival when this was adjusted for the TNM stage. The 3- and 5-year survival rates were: stage Ia [BDSR (100.0% and 100.0%) vs PD (76.9% and 68.4%) (P = 0.226)]; stage Ib [BDSR (55.8% and 32.6%) vs PD (59.3% and 59.3%) (P = 0.942)]; stage IIb [BDSR (19.2% and 19.2%) vs PD (31.9% and 14.2%) (P = 0.669)].
CONCLUSION: BDSR can be justified as an alternative radical operation for patients with middle bile duct in selected patients with no adjacent organ invasion and resection margin is negative.
Bile duct cancer; Segmental resection; Pancreaticoduodenectomy
This study addressed the feasibility and effect of surgical treatment of metachronous periampullary carcinoma after resection of the primary extrahepatic bile duct cancer. The performance of this secondary curative surgery is not well-documented.
We reviewed, retrospectively, the medical records of 10 patients who underwent pancreaticoduodenectomy (PD) for secondary periampullary cancer following extrahepatic bileduct cancer resection from 1995 to 2011.
The mean age of the 10 patients at the second operation was 61 years (range, 45-70 years). The primary cancers were 7 hilar cholangiocarcinomas, 2 middle common bile duct cancers, and one cystic duct cancer. The secondary cancers were 8 distal common bile duct cancers and 2 carcinomas of the ampulla of Vater. The second operations were 6 Whipple procedures and 4 pylorus-preserving pancreaticoduodenectomies. The mean interval between primary treatment and metachronous periampullary cancer was 20.6 months (range, 3.4-36.6 months). The distal resection margin after primary resection was positive for high grade dysplasia in one patient. Metachronous tumor was confirmed by periampullary pathology in all cases. Four of the 10 patients had delayed gastric emptying (n = 2) or pancreatic fistula (n = 2) after reoperation. There were no perioperative deaths. Median survival after PD was 44.6 months (range, 8.5-120.5 months).
Based on the postoperative survival rate, PD may provide an acceptable protocol for resection in patients with metachronous periampullary cancer after resection of the extrahepatic bile duct cancer.
Extrahepatic bile duct; Cholangiocarcinoma; Metachronous neoplasms; Pancreaticoduodenectomy
Prognostic factors for distal bile duct cancer are contentious. This study was conducted to analyze the prognostic factors of distal bile duct cancer after surgery with the aim of identifying those associated with diminished survival.
Two hundred forty-one patients who underwent pylorus-preserving pancreaticoduodenectomy (PPPD) or Whipple procedure in our tertiary hospital from February 1995 to June 2011 were retrospectively analyzed. All patients were pathologically proven to have distal bile duct adenocarcinoma. Postoperative complications, survival, and well-known prognostic factors after resection for distal bile duct cancer were investigated.
Preoperative elevated carbohydrate antigen 19-9 (CA 19-9) level (P = 0.006), positive resection margin (P < 0.001), advanced T stage (P = 0.043), and lymph node metastasis (P = 0.002) were significantly independent worse prognostic indicators by multivariate analysis of resectable distal bile duct cancer.
R0 resection is the most important so that frozen sections should be utilized aggressively during each operation. For the distal bile duct cancer with elevated preoperative CA 19-9 level or advanced stage, further study on postoperative adjuvant treatment may be warranted.
Bile duct cancer; CA-19-9 antigen; Pancreaticoduodenectomy
We investigated the prognostic values of volume-based metabolic parameters by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) in gallbladder carcinoma patients and compared them with other prognostic parameters.
Materials and Methods
We enrolled 44 patients, who were initially diagnosed with gallbladder carcinoma and undergoing 18F-FDG PET/CT. Various metabolic volume-based PET parameters of primary tumors, including maximum and average standardized uptake values (SUVmax, SUVavg), metabolic tumor volume (MTV), and total lesion glycolysis (TLG), were measured in gallbladder carcinoma patients using mediastinal blood pool activity as a threshold SUV for determining the tumor boundaries. Overall survival analysis was performed using the Kaplan-Meier method with PET parameters and other clinical variables. For determining independent prognostic factors, Cox proportional hazards regression analysis was performed.
Of the 44 enrolled patients, cancer- or treatment-related death occurred in 30 (68.2 %). The mean clinical follow-up period was 22.2 ± 10.4 m (range, 0.6-35.9 m). Univariate analysis demonstrated that clinical or pathologic TNM stage (P < 0.001), treatment modality (P < 0.001), MTV (cutoff = 135 cm3, P = 0.001), and TLG (cutoff = 7,090, P < 0.05) were significant prognostic factors. In multivariate analysis, both clinical or pathologic TNM stage [hazard ratio (HR) = 2.019 (I vs II), 21.287 (I vs III), and 24.354 (I vs IV); P = 0.001) and TLG (HR = 2.930; P < 0.05) were independent prognostic factors for predicting overall survival.
In gallbladder cancer, TLG of the primary tumor, a volume-based metabolic parameter, is a significant independent prognostic factor for overall survival in conjunction with the clinical or pathological TNM stage.
Gallbladder carcinoma; Prognosis; 18F-FDG; PET/CT; Metabolic tumor volume; Total lesion glycolysis
Tumors arising from the ampulla of Vater can be benign or malignant. Recently, endoscopic papillectomy has been employed in the management of benign ampulla of Vater tumors; however, surgical resection is the treatment of choice. The aim of this study was to define indications and suggest a role for transduodenal ampullectomy in the management of ampulla of Vater tumors.
We retrospectively reviewed the medical records of 54 patients treated for ampulla of Vater tumors between January 1999 and December 2008.
Twenty-two endoscopic papillectomies and 21 transduodenal ampullectomies were performed. Four patients underwent transduodenal ampullectomy after endoscopic papillectomy due to a recurrent or remnant tumor. Recurrence or a remnant tumor was found in one patient after transduodenal ampullectomy compared to six patients after endoscopic papillectomy. Immediate intraoperative conversion from transduodenal ampullectomy to pancreaticoduodenectomy was performed in five patients based on intraoperative frozen biopsy analysis.
Transduodenal ampullectomy should be performed to treat ampulla of Vater tumors that are unsuitable for endoscopic papillectomy. Transduodenal ampullectomy can serve as an intermediate treatment option between endoscopic papillectomy and pancreaticoduodenectomy in the management of ampulla of Vater tumors.
Ampulla of vater; Transduodenal ampullectomy; Ampullary neoplasm; Endoscopic papillectomy
Solid pancreatic tumors such as pancreatic ductal adenocarcinoma (PDAC), solid pseudopapillary tumor (SPT), and pancreatic endocrine tumor (PET) may occasionally manifest as cystic lesions. In this study, we have put together our accumulated experience with cystic manifestations of various solid tumors of the pancreas.
From 2000 to 2006, 376 patients with pancreatic solid tumor resections were reviewed. Ten (2.66%) of these tumors appeared on radiological imaging studies as cystic lesions. We performed a retrospective review of medical records and pathologic findings of these 10 cases.
Of the ten cases in which solid tumors of the pancreas manifested as cystic lesions, six were PDAC with cystic degeneration, two were SPT undergone complete cystic change, one was cystic PET, and one was a cystic schwannoma. The mean tumor size of the cystic portion in PDAC was 7.3 cm, and three patients were diagnosed as 'pseudocyst' with or without cancer. Two SPT were found incidentally in young women and were diagnosed as other cystic neoplasms. One cystic endocrine tumor was preoperatively suspected as intraductal papillary mucinous neoplasm or mucinous cystic neoplasm.
Cystic changes of pancreas solid tumors are extremely rare. However, the possibility of cystic manifestation of pancreas solid tumors should be kept in mind.
solid; cystic; pancreas; tumor
Prediction of malignancy or invasiveness of branch duct type intraductal papillary mucinous neoplasm (Br-IPMN) is difficult, and proper treatment strategy has not been well established. The authors investigated the characteristics of Br-IPMN and explored its malignancy or invasiveness predicting factors to suggest a scoring formula for predicting pathologic results. From 1994 to 2008, 237 patients who were diagnosed as Br-IPMN at 11 tertiary referral centers in Korea were retrospectively reviewed. The patients' mean age was 63.1 ± 9.2 yr. One hundred ninty-eight (83.5%) patients had nonmalignant IPMN (81 adenoma, 117 borderline atypia), and 39 (16.5%) had malignant IPMN (13 carcinoma in situ, 26 invasive carcinoma). Cyst size and mural nodule were malignancy determining factors by multivariate analysis. Elevated CEA, cyst size and mural nodule were factors determining invasiveness by multivariate analysis. Using the regression coefficient for significant predictors on multivariate analysis, we constructed a malignancy-predicting scoring formula: 22.4 (mural nodule [0 or 1]) + 0.5 (cyst size [mm]). In invasive IPMN, the formula was expressed as invasiveness-predicting score = 36.6 (mural nodule [0 or 1]) + 32.2 (elevated serum CEA [0 or 1]) + 0.6 (cyst size [mm]). Here we present a scoring formula for prediction of malignancy or invasiveness of Br-IPMN which can be used to determine a proper treatment strategy.
Branch Duct Type Intraductal Papillary Mucinous Neoplasm (IPMN); Cyst Size; Mural Nodule; CEA; Malignancy; Invasive Carcinoma
Metastasis to the pancreas is rare, and the benefit of resection for pancreatic metastasis is poorly defined. The aim of this study was to review our experiences of the operative management of metastasis to the pancreas.
Between 1995 and 2009, 11 patients (8 men and 3 women; median age, 54 years) were admitted to our institution with a metachronously metastatic lesion to the pancreas and later underwent pancreatic resection. The clinical features and outcomes of treatments were examined.
The primary cancers were renal cell carcinoma (RCC, n = 7), carcinoid tumor (n = 2), rectal cancer and leiomyosarcoma. Six patients underwent distal pancreatectosplenectomy, 3 pancreaticoduodenectomy and 2 patients underwent enucleation for small RCC. One patient died of metastatic RCC at 53 months after surgery and ten patients remain alive; four patients without disease at 7 to 69 months postoperatively, and the other six with disease at 11 to 68 months. Median postoperative survival of all patients was 34 months.
Patients with a low surgical risk should be considered for pancreatic metastasectomy if curative resection is possible. Primary cancer type, which is associated with survival benefit, would be the best candidate for surgical resection of metastases to the pancreas.
Pancreatic metastasis; Pancreatic metastasectomy; Metastatic pancreatic cancer
Granulocytic sarcoma is a rare extramedullary tumor composed of myeloid progenitor cells. Primary involvement of the biliary tract without evidence of leukemia is exceedingly rare. Here, we report an isolated biliary granulocytic sarcoma in a 30-yr-old man who presented with jaundice, fever, and chill without any evidence of leukemia. However, five months after the diagnosis, he developed acute myelogenous leukemia with multilineage dysplasia and chromosomal abnormality. A rare possibility of biliary granulocytic sarcoma should be considered as a differential diagnosis in patients with obstructive jaundice. A histologic evaluation by aggressive diagnostic intervention is important and may improve prognosis.
Sarcoma, Granulocytic; Leukemia; Jaundice, Obstructive; Bile Ducts
This study was performed to analyze the efficacy of the prophylactic use of octreotide (Novartis, Stein, Switzerland) for pancreatic fistula following a pancreaticoduodenectomy. The medical records of 190 patients who underwent a pancreaticoduodenectomy at the Samsung Medical Center in Seoul, Korea between January 2000 and December 2002 were reviewed. Patients were divided into either the octreotide (n = 81) or control group (n = 109). The octreotide group received subcutaneous injections of 100 µg of octreotide every 12 hours for more than five days after surgery. The control group was not treated with octreotide. The criterion of pancreatic fistula was the drainage of the amylase rich fluid, over 500 U/mL in the three days after surgery. The morbidity and mortality rates were 32.1% and 1.2% in the octreotide group and 31.2% and 0% in the control group, respectively. Pancreatic fistula was the second most common complication (8.4%). In the univariate analysis, octreotide was ineffective in reducing pancreatic fistula (p = 0.26). However, in the multivariate regression analysis, combined gastrectomy (p = 0.018), cellular origin of the disease (p = 0.049), and use of octreotide (p = 0.044) were the risk factors that increased the frequency of pancreatic fistula. Therefore, the routine use of octreotide after a pancreaticoduodenectomy should be avoided until a worldwide consensus is established.
Pancreatic fistula; pancreaticoduodenectomy; octreotide