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Kashima H, Fukuda A, Ueo T, Minami R, Ohana M, Seno H. Intrapancreatic cholangiocarcinoma associated with pancreaticobiliary maljunction without bile duct dilatation 27 years after cholecystectomy. Clin J Gastroenterol 2025:10.1007/s12328-025-02131-0. [PMID: 40232607 DOI: 10.1007/s12328-025-02131-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2025] [Accepted: 04/03/2025] [Indexed: 04/16/2025]
Abstract
A 77-year-old woman was referred to our hospital because of dilatation of the common bile duct. She had undertaken cholecystectomy for abdominal pain and abnormality of gallbladder 27 years before. Computed tomography (CT) and endoscopic ultrasonography (EUS) showed a tumor in the intrapancreatic bile duct and dilatation of the extrahepatic and intrahepatic bile ducts. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a tumor in the intrapancreatic bile duct and pancreaticobiliary maljunction (PBM). Previous CT did not show dilatation of the bile duct, indicating that she had PBM without biliary dilatation. Biopsy of the tumor revealed adenocarcinoma, and she was diagnosed with distal cholangiocarcinoma. She undertook pancreatoduodenectomy. Pancreatography of the surgically resected specimen confirmed the connection between the main pancreatic duct and common bile duct. The final pathological diagnosis of the tumor was intrapancreatic papillary adenocarcinoma, pT2N0M0, stage IB. Recurrence has not been observed for 6 years after surgery. For PBM without bile duct dilatation, prophylactic cholecystectomy is recommended to prevent gallbladder carcinoma; however, biliary diversion surgery is controversial and is not generally recommended. Our case underscores that PBM without bile duct dilatation has a risk of extrahepatic biliary cancer and that careful follow-up is important after prophylactic cholecystectomy for a long period.
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Affiliation(s)
- Hirotaka Kashima
- Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara Town, Shogoin, Sakyo-ku, Kyoto, Kyoto, 606-8507, Japan.
| | - Akihisa Fukuda
- Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara Town, Shogoin, Sakyo-ku, Kyoto, Kyoto, 606-8507, Japan
| | - Taro Ueo
- Department of Gastroenterology, Tenri Hospital, 200 Mishima Town, Tenri, Nara, 632-8552, Japan
| | - Ryuki Minami
- Department of Gastroenterology, Tenri Hospital, 200 Mishima Town, Tenri, Nara, 632-8552, Japan
| | - Masaya Ohana
- Department of Gastroenterology, Tenri Hospital, 200 Mishima Town, Tenri, Nara, 632-8552, Japan
| | - Hiroshi Seno
- Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara Town, Shogoin, Sakyo-ku, Kyoto, Kyoto, 606-8507, Japan
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Quaglia A, Roberts EA, Torbenson M. Developmental and Inherited Liver Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2024:122-294. [DOI: 10.1016/b978-0-7020-8228-3.00003-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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Vega EA, Mellado S, Salehi O, Freeman R, Conrad C. Treatment of Resectable Gallbladder Cancer. Cancers (Basel) 2022; 14:1413. [PMID: 35326566 PMCID: PMC8945892 DOI: 10.3390/cancers14061413] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2022] [Revised: 02/18/2022] [Accepted: 03/06/2022] [Indexed: 02/04/2023] Open
Abstract
Gallbladder cancer (GBC) is the most common biliary tract cancer worldwide and its incidence has significant geographic variation. A unique combination of predisposing factors includes genetic predisposition, geographic distribution, female gender, chronic inflammation, and congenital developmental abnormalities. Today, incidental GBC is the most common presentation of resectable gallbladder cancer, and surgery (minimally invasive or open) remains the only curative treatment available. Encouragingly, there is an important emerging role for systemic treatment for patients who have R1 resection or present with stage III-IV. In this article, we describe the pathogenesis, surgical and systemic treatment, and prognosis.
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Affiliation(s)
- Eduardo A. Vega
- Department of Surgery, St. Elizabeth’s Medical Center, Boston University School of Medicine, Boston, MA 02135, USA; (E.A.V.); (O.S.); (R.F.)
| | | | - Omid Salehi
- Department of Surgery, St. Elizabeth’s Medical Center, Boston University School of Medicine, Boston, MA 02135, USA; (E.A.V.); (O.S.); (R.F.)
| | - Richard Freeman
- Department of Surgery, St. Elizabeth’s Medical Center, Boston University School of Medicine, Boston, MA 02135, USA; (E.A.V.); (O.S.); (R.F.)
| | - Claudius Conrad
- Department of Surgery, St. Elizabeth’s Medical Center, Boston University School of Medicine, Boston, MA 02135, USA; (E.A.V.); (O.S.); (R.F.)
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Suzuki M, Minowa K, Isayama H, Shimizu T. Acute recurrent and chronic pancreatitis in children. Pediatr Int 2021; 63:137-149. [PMID: 32745358 DOI: 10.1111/ped.14415] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2020] [Revised: 06/27/2020] [Accepted: 07/28/2020] [Indexed: 12/14/2022]
Abstract
Acute recurrent pancreatitis (ARP) is defined as two distinct episodes of acute pancreatitis (AP), whereas chronic pancreatitis (CP) is caused by persistent inflammation of the pancreas. In children they are caused by genetic mutations, autoimmune pancreatitis, congenital pancreatic abnormalities, and other conditions. Acute recurrent pancreatitis is frequently a precursor to CP, and both are thought to be on the same disease continuum. In particular, genetic factors are associated with early progression of ARP to CP. The diagnosis of CP, as in AP, is based on clinical findings, biochemical tests, and imaging studies. Findings of exocrine pancreatic dysfunction are also important in the diagnosis of CP. A step-up strategy has become increasingly standard for the treatment of patients with CP. This strategy starts with endoscopic treatment, such as pancreatic sphincterotomy and stenting, and progresses to surgery should endoscopic therapy fail or prove technically impossible. Non-opioid (e.g. ibuprofen / naproxen) and opioid (e.g. oxycodone) forms of analgesia are widely used in pediatric patients with AP or CP, whereas pancreatic enzyme replacement therapy may be beneficial for patients with abdominal pain, steatorrhea, and malnutrition. Despite the disparity in the age of onset, pediatric CP patients display some similarities to adults in terms of disease progress. To reduce the risk of developing pancreatic exocrine inefficiency, diabetes and pancreatic cancer in the future, clinicians need to be aware of the current diagnostic approach and treatment methods for ARP and CP and refer them to a pediatric gastroenterologist in a timely manner.
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Affiliation(s)
- Mitsuyoshi Suzuki
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
| | - Kei Minowa
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
| | - Hiroyuki Isayama
- Department of Gastroenterology, Juntendo University Graduate School of Medicine, Tokyo, Japan
| | - Toshiaki Shimizu
- Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
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Iwasaki T, Otsuka Y, Miyata Y, Einama T, Tsujimoto H, Ueno H, Ogata S, Kishi Y. Intracholecystic papillary neoplasm arising in a patient with pancreaticobiliary maljunction: a case report. World J Surg Oncol 2020; 18:292. [PMID: 33168026 PMCID: PMC7654008 DOI: 10.1186/s12957-020-02072-7] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2020] [Accepted: 10/27/2020] [Indexed: 02/07/2023] Open
Abstract
Background Pancreaticobiliary maljunction (PBM) is a congenital abnormality in which the pancreatic and biliary ducts join anatomically outside the duodenal wall resulting in the regurgitation of pancreatic juice into the biliary tract (pancreatobiliary reflux). Persistent pancreatobiliary reflux causes injury to the epithelium of the biliary tract and promotes the risk of biliary cancer. Intracholecyctic papillary neoplasm (ICPN) has been highlighted in the context of a cholecystic counterpart of intraductal papillary mucinous neoplasm of the pancreas and the bile duct, but the tumorigenesis of ICPNs remains unclear. Case presentation A 52-year-old Japanese woman was referred for the assessment of dilation of the bile duct. Computed tomography which revealed an enhanced mass in the gallbladder and endoscopic retrograde cholangiopancreatography confirmed that the confluence of the main pancreatic duct and extrahepatic bile duct (EHBD) was located outside the duodenal wall. Under the diagnosis of gallbladder cancer with PBM, cholecystectomy with full thickness dissection, EHBD resection, lymph node dissection, and hepaticojejunostomy were performed. Macroscopic examination of the resected specimen showed that the cystic duct was dilated and joined into the EHBD just above its confluence with the pancreatic duct, and the inflamed change of non-tumorous mucosa of gallbladder indicating that there was considerable mucosal injury due to pancreatobiliary reflux to the gallbladder. Histopathological examination revealed that the gallbladder tumor was a gastric-type ICPN with non-invasive component. Either KRAS gene mutation or p53 protein expression that were known to be associated with the carcinogenesis of biliary cancer under the condition of pancreatobiliary reflux was not detected in the tumor cells of ICPN. Conclusion The present case might suggest that there was no association between PBM and ICPN. To reveal the tumorigenesis of ICPN and its attribution to pancreatobiliary reflux, however, further study is warranted.
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Affiliation(s)
- Toshimitsu Iwasaki
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan.
| | - Yasuhiro Otsuka
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Yoichi Miyata
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Takahiro Einama
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Hironori Tsujimoto
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Hideki Ueno
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Sho Ogata
- Department of Pathology and Laboratory Medicine, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-0042, Japan
| | - Yoji Kishi
- Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
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Zhu L, Xiong J, Lv Z, Liu J, Huang X, Xu W. Type C Pancreaticobiliary Maljunction Is Associated With Perforated Choledochal Cyst in Children. Front Pediatr 2020; 8:168. [PMID: 32363172 PMCID: PMC7181898 DOI: 10.3389/fped.2020.00168] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2020] [Accepted: 03/25/2020] [Indexed: 12/30/2022] Open
Abstract
Background: Perforation of a choledochal cyst (CC) is not rare, but the pathogenesis of spontaneous perforation has not been established. Pancreaticobiliary maljunction (PBM) is commonly seen in association with choledochal cyst. To explore the relationship between PBM and perforated CC, a retrospective study was conducted. Methods: We analyzed all the patients with CC who underwent surgery in our hospital from 2014.06.01 to 2018.12.31. All patients were divided into two groups: group 1 were patients with perforated CC, and group 2 were patients with non-perforated CC. We recalled all the patients records to identify types of PBM. PBM was divided into four types [(A) stenotic type, (B) non-stenotic type, (C) dilated channel type, and (D) complex type] according to the classification proposed by the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2015. Results: There were 186 patients with CC in all, and 116 patients showed PBM. Twenty patients in group 1 and 96 patients in group 2. There was an extremely higher percentage of type C PBM in group 1 than in group 2 (60 and 17.7%, respectively). More fusiform dilatation cases were found in group 1 (70%) than in group 2 (58.3%). Also there were more type C PBM in fusiform cases and type A PBM were frequently seen in cystic cases (P < 0.01). Conclusions: We found that Type C PBM and fusiform common bile duct maybe relate to the perforation of choledochal cyst. Patients with type C PBM and fusiform common bile duct should be treated more proactively, preferably before they perforate.
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Affiliation(s)
- Linlin Zhu
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Jing Xiong
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Zhibao Lv
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Jiangbin Liu
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Xiong Huang
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Weijue Xu
- Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
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Baison GN, Bonds MM, Helton WS, Kozarek RA. Choledochal cysts: Similarities and differences between Asian and Western countries. World J Gastroenterol 2019; 25:3334-3343. [PMID: 31341359 PMCID: PMC6639560 DOI: 10.3748/wjg.v25.i26.3334] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/20/2019] [Revised: 05/05/2019] [Accepted: 05/31/2019] [Indexed: 02/06/2023] Open
Abstract
Choledochal cysts (CCs) are rare bile duct dilatations, intra-and/or extrahepatic, and have higher prevalence in the Asian population compared to Western populations. Most of the current literature on CC disease originates from Asia where these entities are most prevalent. They are thought to arise from an anomalous pancreaticobiliary junction, which are congenital anomalies between pancreatic and bile ducts. Some similarities in presentation between Eastern and Western patients exist such as female predominance, however, contemporary studies suggest that Asian patients may be more symptomatic on presentation. Even though CC disease presents with an increased malignant risk reported to be more than 10% after the second decade of life in Asian patients, this risk may be overstated in Western populations. Despite this difference in cancer risk, management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer.
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Affiliation(s)
- George N Baison
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - Morgan M Bonds
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - William S Helton
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
| | - Richard A Kozarek
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA 98101, United States
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Hori T. Comprehensive and innovative techniques for laparoscopic choledocholithotomy: A surgical guide to successfully accomplish this advanced manipulation. World J Gastroenterol 2019; 25:1531-1549. [PMID: 30983814 PMCID: PMC6452235 DOI: 10.3748/wjg.v25.i13.1531] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2019] [Revised: 03/05/2019] [Accepted: 03/11/2019] [Indexed: 02/06/2023] Open
Abstract
Surgeries for benign diseases of the extrahepatic bile duct (EHBD) are classified as lithotomy (i.e., choledocholithotomy) or diversion (i.e., choledochojejunostomy). Because of technical challenges, laparoscopic approaches for these surgeries have not gained worldwide popularity. The right upper quadrant of the abdomen is advantageous for laparoscopic procedures, and laparoscopic choledochojejunostomy is safe and feasible. Herein, we summarize tips and pitfalls in the actual procedures of choledocholithotomy. Laparoscopic choledocholithotomy with primary closure of the transductal incision and transcystic C-tube drainage has excellent clinical outcomes; however, emergent biliary drainage without endoscopic sphincterotomy and preoperative removal of anesthetic risk factors are required. Elastic suture should never be ligated directly on the cystic duct. Interrupted suture placement is the first choice for hemostasis near the EHBD. To prevent progressive laceration of the EHBD, full-layer interrupted sutures are placed at the upper and lower edges of the transductal incision. Cholangioscopy has only two-way operation; using dedicated forceps to atraumatically grasp the cholangioscope is important for smart maneuvering. The duration of intraoperative stone clearance accounts for most of the operative time. Moreover, dedicated forceps are an important instrument for atraumatic grasping of the cholangioscope. Damage to the cholangioscope requires expensive repair. Laparoscopic approach for choledocholithotomy involves technical difficulties. I hope this document with the visual explanation and literature review will be informative for skillful surgeons.
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Affiliation(s)
- Tomohide Hori
- Department of Hepato-Biliary-Pancreatic Surgery, Kyoto University Graduate School of Medicine, Kyoto 606-8507, Japan
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Quaglia A, Roberts EA, Torbenson M. Developmental and Inherited Liver Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2018:111-274. [DOI: 10.1016/b978-0-7020-6697-9.00003-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC). World J Surg 2017; 41:538-545. [PMID: 27620132 DOI: 10.1007/s00268-016-3684-x] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND Pancreaticobiliary maljunctions (PBMs) are congenital anomalies of the junction between pancreatic and bile ducts, frequently associated with bile duct cyst (BDC). BDC is congenital biliary tree diseases that are characterized by distinctive dilatation types of the extra- and/or intrahepatic bile ducts. Todani's types I and IVa, in which dilatation involves principally the main bile duct, are the most frequent. PBM induces pancreatic juice reflux into the biliary tract that is supposed to be one of the main factors of biliary cancer degeneration, although the diagnostic criteria of PBM that can be either morphological and/or functional are not well defined especially in Western series. OBJECTIVE The aim of this study was to assess the relative prevalence of PBM in BDC in a large European multicenter study, to analyze the characteristics of PBM and try to propose diagnostic criteria of PBMs based on morphological and/or functional criteria and define the positive, negative predictive values, sensibility and specificity of either criteria. RESULTS From 1975 to 2012, 263 patients with BDC were analyzed. Among them, 190 (72.2 %) were considered to present PBM. Types I and IVa had a similar rate of PBM association. According to the "AFC classification," 57.2 % had a C-P type, 34.5 % a P-C type and 8.3 % a complex type ("anse-de-seau"). The median length of the common channel in patients with PBM was 15.8 ± 6.8 mm (range 5-40 mm). The median intrabiliary amylase and lipase levels were 65,249 and 172,104 UI/L, respectively. For the diagnostic of PBM, a common channel length of more than 8 mm and an intrabiliary amylase level superior to 8000 UI/L were associated with a predictive positive value and a specificity of more than 90 %. Synchronous biliary cancer had an incidence of 8.7 % in all patients with BDC and PBM 11.1 % in adults. Compared to type IV, the type I BDC was associated with statistically more cancer patients in the presence of PBM. CONCLUSIONS Characteristics of PBM associated with BDC in Western population are quite close to reported Eastern series. The results suggest considering both the intrabiliary value of amylase >8000 UI/L and a length of a common channel >8 mm as appropriate values for positive diagnosis of PBM.
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Free J, Wang F, Williams N, Gundara JS, Staerkle RF, Hugh TJ, Samra JS. Gallbladder mucosal lesions associated with high biliary amylase irrespective of pancreaticobiliary maljunction. ANZ J Surg 2017; 88:E517-E521. [PMID: 28782883 DOI: 10.1111/ans.14136] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2017] [Revised: 06/02/2017] [Accepted: 06/05/2017] [Indexed: 01/03/2023]
Abstract
BACKGROUND Previous studies have focused on the presence of reflux in selected cohorts with pancreaticobiliary maljunction (PBM), but little is known regarding the wider incidence of occult reflux and associated mucosal changes. We aimed to correlate gallbladder mucosal abnormalities with objective evidence of PBM and occult pancreaticobiliary reflux (PBR) in an Australian population undergoing cholecystectomy. METHODS Patients undergoing cholecystectomy between September 2010 and September 2012 were eligible for inclusion. Demographic and pre-operative clinical data were collated and entered into a pre-defined database. Operative cholangiograms were routinely performed and the presence of PBM noted. Gallbladder bile samples were analysed for bilirubin (<20 µmol/L), amylase (<100 U/L) and lipase (<70 U/L) levels. Evidence of PBR was correlated with gallbladder mucosal findings. RESULTS A total of 305 cholecystectomies were performed for biliary colic (73%), choledocholithiasis (9%), cholecystitis (8.4%) and pancreatitis (6.4%). A total of 12.7% had cholangiographic evidence of PBM and 11.9% possessed gallbladder mucosal changes. Overall, 7.7% had increased biliary amylase, which was associated with significantly higher rates of gallbladder intestinal metaplasia (33% versus 8.6%; P = 0.012). Elevated biliary amylase was also higher in patients with prior pancreatitis (P = 0.02) or choledocholithiasis (P < 0.01). The presence of PBM did not predict for the presence of PBR. CONCLUSION PBR is associated with an increased frequency of gallbladder mucosal metaplasia, irrespective of the presence of PBM. Objectively identified reflux represents an additional indication for cholecystectomy but the long-term consequences for extra-hepatic biliary malignancy remain unknown and warrant further investigation. Methods of objectively identifying PBR pre-operatively require further investigation.
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Affiliation(s)
- Jason Free
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Frank Wang
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Nick Williams
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Justin S Gundara
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Ralph F Staerkle
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Thomas J Hugh
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
| | - Jaswinder S Samra
- Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital and North Shore Private Hospital, The University of Sydney, Sydney, New South Wales, Australia
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Huh CW, Kim HW, Yi SW, Lee DK, Lee SJ. Common bile duct stones associated with pancreatobiliary reflux and disproportionate bile duct dilatation. Medicine (Baltimore) 2017; 96:e7701. [PMID: 28834874 PMCID: PMC5571996 DOI: 10.1097/md.0000000000007701] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Occult pancreatobiliary reflux (PBR) in patients with a normal pancreatobiliary junction has been studied by various methods, but the exact etiology, mechanisms, and implications of this reflux have not yet been clarified. The aim of this study was to investigate the degree of PBR and patterns of biliary ductal dilatation in patients with acute calculous cholangitis by endoscopic retrograde cholangiopancreatography (ERCP).We retrospectively evaluated the degree of PBR and pattern of bile duct dilatation in patients with acute calculous cholangitis due to distal CBD (common bile duct) stones (Group A) as compared with patients with malignant CBD obstruction due to distal CBD cancer (Group B). All related data were prospectively collected. Bile juice was aspirated at the proximal CBD for measurement of biliary amylase and lipase before the injection of contrast dye. The diameters of the CBD and the peripheral intrahepatic duct (IHD) were calculated after contrast dye injection. Patients with pancreatobiliary maljunction and/or gallstone pancreatitis were excluded from the study.ERCP was performed on 33 patients with calculous cholangitis (Group A) and 12 patients with malignant CBD obstruction (Group B). Mean levels of bile amylase and lipase were significantly higher (P < .05) in group A (1387 and 6737 U/l, respectively) versus those in group B (32 and 138 U/l, respectively). Thirty patients in group A (90.9%) showed disproportionate dilatation (i.e., CBD was and IHD was not dilated), whereas only 4 patients in group B (33%) showed disproportionate dilatation.The results of this study suggest that patients with calculous cholangitis exhibit PBR that is associated with disproportionate bile duct dilatation.
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Affiliation(s)
- Cheal Wung Huh
- Division of Gastroenterology, Department of Internal Medicine, College of Medicine, Incheon St. Mary's Hospital, the Catholic University of Korea, Incheon
| | - Hee Wook Kim
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Seung Woo Yi
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Dong Ki Lee
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Se Joon Lee
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
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Ikegame K, Takano A, Watanabe H, Yamamoto A, Miyasaka Y, Furuya K, Nakada H, Sugai H, Yasutome M, Inoue M, Hada M, Nakagomi H, Omata M, Oyama T. Biliary cancer developed after the reparative surgery for congenital choledochal cyst: a case report and review of the literature. Int Cancer Conf J 2017; 6:43-49. [PMID: 31149469 PMCID: PMC6498380 DOI: 10.1007/s13691-016-0270-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2016] [Accepted: 11/06/2016] [Indexed: 11/26/2022] Open
Abstract
The incidence of bile duct cancer developed in the patients with congenital choledochal cysts (CC) with a history of reparative surgery is not well known. We experienced a case developed choleductal cancer 45 years after reparative surgery. She underwent reparative surgery with cyst excision and hepatic bile duct duodenal anastomosis at 1 year of age. She developed the symptoms of jaundice, anorexia, and dull pain in the right upper part of the abdomen at 47 years of age. The carcinoma arose from the dilated proximal bile duct anastomosed with the duodenum. Cholestasis and regurgitation of duodenal fluids seemed to have influenced the development of cancer in this patient. We additionally reviewed seven cohort studies concerning the incidence of biliary carcinoma after surgery for congenital choledocal cysts. The incidence of biliary cancer developed after surgery was 2.2 ± 2.5 (ranged 0-6.5)%. We also reviewed 33 Japanese case reports cited in Japan MEDLINE from 1986 to 2015. Regarding the CC types according to the Totani's classification, 12 were type I and 14 type IVa, 1 was type II and 6 were unknown type. The rate of coexistence of pancreaticobiliary maljunction (PBM) was 92%, (22/24; other 9 cases were not documented), and biliary cancer arose from the proximal stump of the reparative surgery in 68% (17/25) and from the distal stump in 32% (8/25) of cases. These findings suggested that the reflux of pancreatic juice due to PBM only partially explained the carcinogenesis of CCs. Intensive follow-up of such patients throughout their lives is necessary to avoid cancer death even after standard reparative surgery.
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Affiliation(s)
- Kou Ikegame
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Atsushi Takano
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hideki Watanabe
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Atsushi Yamamoto
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Yoshiaki Miyasaka
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Kazushige Furuya
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Haruka Nakada
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hidemitsu Sugai
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Michiya Yasutome
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masayuki Inoue
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masao Hada
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Hiroshi Nakagomi
- Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506 Japan
| | - Masao Omata
- Department of Internal Medicine, Yamanashi Prefectural Central Hospital, Kofu, Japan
| | - Toshio Oyama
- Department of Pathology, Yamanashi Prefectural Central Hospital, Kofu, Japan
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Karami H, Dabirian M. A Review on Acute Pediatric Pancreatitis. JOURNAL OF PEDIATRICS REVIEW 2016. [DOI: 10.17795/jpr-5425] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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15
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Hamada Y, Ando H, Kamisawa T, Itoi T, Urushihara N, Koshinaga T, Saito T, Fujii H, Morotomi Y. Diagnostic criteria for congenital biliary dilatation 2015. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2016; 23:342-6. [PMID: 26996969 DOI: 10.1002/jhbp.346] [Citation(s) in RCA: 66] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/07/2016] [Accepted: 03/17/2016] [Indexed: 12/13/2022]
Abstract
BACKGROUND The Diagnostic Criteria for Pancreaticobiliary Maljunction 2013 were published by the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2014. The committee of JSGPM for diagnostic criteria for pancreaticobiliary maljunction has established the standard diameter of the bile duct, and a definition of dilatation of the bile duct was proposed in 2014. METHODS The committee of JSGPM prepared the diagnostic criteria for congenital biliary dilatation in 2014, and a final revised version was approved in 2015. RESULTS Congenital biliary dilatation is defined as a congenital malformation involving both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. However, cases associated with intrahepatic bile duct dilatation can also be included. Various kinds of pathological conditions can occur on hepatobiliary systems and pancreas by bile duct dilatation and pancreaticobiliary maljunction. For a diagnosis of congenital biliary dilatation, both abnormal dilatation of the bile duct and pancreaticobiliary maljunction must be evident by either imaging test or anatomical examination. Acquired or secondary dilatation of the bile duct by obstruction due to biliary stones or malignancy should be strictly excluded. CONCLUSION Diagnostic criteria for congenital biliary dilatation 2015 were established from Japan representing a world first.
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Affiliation(s)
- Yoshinori Hamada
- Division of Pediatric Surgery, Department of Surgery, Kansai Medical University, Hirakata City, Osaka, Japan.
| | | | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Naoto Urushihara
- Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan
| | - Tsugumichi Koshinaga
- Division of Pediatric Surgery, Department of Surgery, Nihon University School of Medicine, Tokyo, Japan
| | - Takeshi Saito
- Department of Pediatric Surgery, Chiba University School of Medicine, Chiba, Japan
| | - Hideki Fujii
- First Department of Surgery, University of Yamanashi, Kofu, Japan
| | - Yoshiki Morotomi
- Division of Pediatric Surgery, Department of Surgery, Osaka City University School of Medicine, Osaka, Japan
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Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat 2016; 38:1053-1060. [DOI: 10.1007/s00276-016-1669-y] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2015] [Accepted: 03/10/2016] [Indexed: 02/07/2023]
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Saito T, Terui K, Mitsunaga T, Nakata M, Ono S, Mise N, Yoshida H. Evidence for viral infection as a causative factor of human biliary atresia. J Pediatr Surg 2015; 50:1398-404. [PMID: 25979202 DOI: 10.1016/j.jpedsurg.2015.04.006] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2014] [Revised: 04/07/2015] [Accepted: 04/20/2015] [Indexed: 01/01/2023]
Abstract
OBJECTIVES To explore the evidence for viral infections triggering human biliary atresia (BA) by reviewing archival original articles that analyzed human samples via polymerase chain reaction (PCR) experiments, considering the recent experimental trend of extensive use of rotaviral BA animal models. METHODS A PubMed search retrieved original articles that reported the results of PCR experiments for detecting viral DNA or RNA in patient samples as proof of past infection. Search terms included the often-debated DNA or RNA viruses and BA. Special focus was directed toward PCR analyses that targeted reovirus and rotavirus, where PCR accuracy, specimen characteristics and their interpretations were compared. RESULTS Nineteen studies were conducted on 16 different kinds of viruses using PCR, with 5 studies on reovirus, 3 on rotavirus, 10 on cytomegalovirus, 5 on Epstein-Barr virus, 4 on parvovirus B19, and so on. Among the papers suggesting a possible viral link to only BA, there was no study on reovirus, 1 on rotavirus, 3 on cytomegalovirus, 1 on EB virus, and 1 on papillomavirus. Of the 6 PCR studies on Reoviridae, 3 on reovirus and 2 on rotavirus were evaluated rigorously for experimental accuracy, including their sensitivity. Two research groups analyzed preoperative stool samples in addition to generic hepatobiliary tissue obtained at surgery. Sample collection timing varied widely, with storage period prior to PCR experimentation not revealed in most reports on Reoviridae. CONCLUSION Although a considerable number of PCR studies have sought to clarify a viral role in the pathogenesis of BA using human samples, the findings have been contradictory and have not succeeded in achieving an obvious differentiation between causative and accidental infection of the focused virus. Reproducible and convincing evidence for a causative Reoviridae infection has been lacking based on objective data from highly sensitive PCR experiments. Even though the possibility remains of viral disappearance at the timing of collection, to avoid further ambiguous interpretations of PCR results, rigorous and meticulous collection of large numbers of specimens at carefully planned timing, along with a strictly adjusted and finely tuned PCR system, is strongly recommended for obtaining more reliable and consistent results.
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Affiliation(s)
- Takeshi Saito
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan.
| | - Keita Terui
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Tetsuya Mitsunaga
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Mitsuyuki Nakata
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Sachie Ono
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Naoko Mise
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Hideo Yoshida
- Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chiba, Japan
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Tang L, Wang XD, Xu JY, Wang J, Huang SG, Chen F. Anatomical and Morphological Features of the Fetal Human Pancreaticobiliary Ductal Union. Fetal Pediatr Pathol 2015; 34:355-60. [PMID: 26417940 DOI: 10.3109/15513815.2015.1087608] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
To investigate pancreaticobiliary ductal anatomy during developmental stages, gallbladders, common bile ducts, pancreatic ducts and their interface with the duodenum were studied in 36 human fetuses between 4-6 weeks postconceptual age were studied. For histological examination, sections were cut continuously from the paraffin-embedded tissue block and stained with hematoxylin and eosin. The expression of proliferating cell nuclear antigen in the gallbladder was examined with immunohistochemistry. Among 36 cases, three shapes of the greater duodenal papilla were found: hemispheroid (58.1%), circular cylinder (25%), and flat shape (16.9%). For the location of the greater duodenal papillas, more than half (69.4%) of the cases were in the middle descendant duodenum. Seven cases (19.4%) were in the lower descendant duodenum. Three cases (8.3%) were in the upper descendant duodenum, and one (2.9%) was in the distal descending part of duodenum. There were four types of the pancreaticobiliary ductal union: "Y" in 24 cases(66.7%), "U" in 4 cases (11.1%),"V" in 7 cases (19.4%), and pancreaticobiliary maljunction in 1 case (2.8%). For patients with congenital bile duct dilation and Biliary cancer, the positive cells of proliferating cell nuclear antigen were increased significantly (P < 0.05). Different types in pancreaticobiliary ductal union investigated in this study may provide clues for pathogenesis and clinical treatment of pancreaticobiliary maljunction.
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Affiliation(s)
- Lin Tang
- a Nanjing University of Chinese Medicine , Nanjing , China
| | - Xing-Dong Wang
- b Soochow University Affiliated Children's Hospital , Suzhou , China
| | - Jian-Ya Xu
- b Soochow University Affiliated Children's Hospital , Suzhou , China
| | - Jian Wang
- b Soochow University Affiliated Children's Hospital , Suzhou , China
| | - Shun-Gen Huang
- b Soochow University Affiliated Children's Hospital , Suzhou , China
| | - Feng Chen
- c Shanghai Jiao Tong University Affiliated Sixth People's Hospital , Shanghai , China
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Suzuki M, Sai JK, Shimizu T. Acute pancreatitis in children and adolescents. World J Gastrointest Pathophysiol 2014; 5:416-26. [PMID: 25400985 PMCID: PMC4231506 DOI: 10.4291/wjgp.v5.i4.416] [Citation(s) in RCA: 52] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2014] [Revised: 04/09/2014] [Accepted: 07/18/2014] [Indexed: 02/06/2023] Open
Abstract
In this Topic Highlight, the causes, diagnosis, and treatment of acute pancreatitis in children are discussed. Acute pancreatitis should be considered during the differential diagnosis of abdominal pain in children and requires prompt treatment because it may become life-threatening. The etiology, clinical manifestations, and course of acute pancreatitis in children are often different than in adults. Therefore, the specific features of acute pancreatitis in children must be considered. The etiology of acute pancreatitis in children is often drugs, infections, trauma, or anatomic abnormalities. Diagnosis is based on clinical symptoms (such as abdominal pain and vomiting), serum pancreatic enzyme levels, and imaging studies. Several scoring systems have been proposed for the assessment of severity, which is useful for selecting treatments and predicting prognosis. The basic pathogenesis of acute pancreatitis does not greatly differ between adults and children, and the treatments for adults and children are similar. In large part, our understanding of the pathology, optimal treatment, assessment of severity, and outcome of acute pancreatitis in children is taken from the adult literature. However, we often find that the common management of adult pancreatitis is difficult to apply to children. With advances in diagnostic techniques and treatment methods, severe acute pancreatitis in children is becoming better understood and more controllable.
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Roa I, Ibacache G, Muñoz S, de Aretxabala X. Gallbladder cancer in Chile: Pathologic characteristics of survival and prognostic factors: analysis of 1,366 cases. Am J Clin Pathol 2014; 141:675-82. [PMID: 24713738 DOI: 10.1309/ajcpqt3eln2bbcka] [Citation(s) in RCA: 44] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
OBJECTIVES To explore gallbladder cancer (GBC), the second leading cause of cancer-related death in women in Chile. METHODS Analysis of macroscopic and microscopic variables, morphometry, and survival in 1,366 patients with GBC. RESULTS Patients comprised 1,138 women and 228 men; diagnoses included 213 (15.6%) cases of mucosal carcinoma, 132 (9.7%) cases of muscular carcinoma, 316 (23.1%) cases of subserosal carcinoma, 382 (28.0%) cases of serosal carcinoma, and 323 (23.6%) cases beyond the serosa. Women older than 55 years with a gallbladder length greater than 9.5 cm had a five-times-greater relative risk of cancer. Those with a gallbladder wall thickness less than 7 mm had a better 5-year survival rate than those with a gallbladder wall thickness greater than 10 mm (P = .0001). Patients who had cholesterolosis of the gallbladder had 9.2 times less probability of having cancer. The infiltration level of the gallbladder wall was the most important independent prognostic factor (P < .001), followed by differentiation and lymphatic involvement (P < .001 and P = .05, respectively). Vascular infiltration had a mortality rate of 100%. CONCLUSIONS Morphologic features are strongly associated with the prognosis of GBC and must be taken into consideration when supplementary treatment is recommended.
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Affiliation(s)
- Iván Roa
- Molecular Laboratory, Creative Bioscience, Santiago, Chile
- Histopathology and Cytopathology Diagnosis Center, Temuco, Chile
| | - Gilda Ibacache
- Histopathology and Cytopathology Diagnosis Center, Temuco, Chile
| | - Sergio Muñoz
- CIGES Faculty of Medicine, Universidad de la Frontera, Temuco, Chile
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Abstract
INTRODUCTION Gallbladder cancer has a poor prognosis, with a reported 5-year survival of 5%. The prognosis improves when an R0 resection is feasible, but an early diagnosis is rare. The aim of the present study is to analyze the different conditions associated with gallbladder carcinomas and to report the main prognostic factors for these tumors to enable prevention. MATERIALS AND METHODS From 1986 to 2012, 75 patients were found to have gallbladder cancer during the study of 2942 patients affected by biliary tract diseases; 34 of these patients had gallbladder and gallstones, and had been subjected to bile analysis. Pancreatobiliary reflux was studied by biliary trypsin and C-Ki-ras genes were analyzed in 11 cases. RESULTS Gallstones were found in 72 of 75 gallbladder cancer patients; in particular, large gallstones were associated with 88.88% of squamous-cell carcinoma, 68.2% of adenocarcinoma, and never with papillary adenocarcinoma. Pancreatobiliary reflux was associated with papillary adenocarcinoma in 100% of cases, but seldom with squamous cell carcinoma. C-Ki-ras mutations were found in 100% of patients with papillary carcinoma. DISCUSSION AND CONCLUSION R0 resection in in-situ cancer has the best prognosis. Preventive cholecystectomy should be performed in high-risk patients (gallstones larger 3 cm; adenomatous polyps>1 cm; pancreatobiliary reflux, porcelain gallbladder, segmental adenomyomatosis, xanthogranulomatous cholecystitis). The histological stratification of gallbladder cancer should be carried out before starting further studies because squamous-cell carcinoma, adenocarcinoma, and papillary carcinoma are associated with different risk factors and genetic mutations and have different responsiveness to chemotherapies.
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Cheng L, Tian F, Zhao T, Pang Y, Luo Z, Ren J. Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases. BMC Gastroenterol 2013; 13:153. [PMID: 24156788 PMCID: PMC4015270 DOI: 10.1186/1471-230x-13-153] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/06/2013] [Accepted: 10/15/2013] [Indexed: 02/07/2023] Open
Abstract
Background Annular pancreas (AP) concurrent with pancreaticobiliary maljunction (PBMJ), an unusual coexisted congenital anomaly, often presented symptoms and subjected surgical treatment at the early age of life. We reported the first adult case of concurrent AP with PBMJ presented with symptoms until his twenties, and performed a literature review to analyze the clinicopathological features of such cases comparing with its pediatric counterpart. Case presentation The main clinical features of this case were abdominal pain and increased levels of plasma amylase as well as liver function test. A complete type of annular pancreas with duodenal stenosis was found, and dilated common bile duct with high confluence of pancreaticobiliary ducts was also observed. Meanwhile, extremely high levels of bile amylase were detected both in common bile duct and gallbladder. The patient received duodenojejunostomy (side-to-side anastomosis) as well as choledochojejunostomy (Roux-en-Y anastomosis), adnd was discharged in a good condition. Conclusion AP concurrent with PBMJ usually presents as duodenal obstruction in infancy, while manifests as pancreatitis in adulthood. Careful long-term follow-up is required for children with AP considering its association with PBMJ which would induce various intractable pathologic conditions in the biliary tract and pancreas.
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Affiliation(s)
| | - Fuzhou Tian
- Department of General Surgery, General Hospital of Chengdu Military Command, Chengdu, Sichuan Province, People's Republic of China.
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Kim SH, Kim HW, Kang DH, Kim MD, Lee JH, Lee JH, Kim BG, Park JH. [A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2013; 60:123-7. [PMID: 22926125 DOI: 10.4166/kjg.2012.60.2.123] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.
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Affiliation(s)
- Suk Hun Kim
- Department of Internal Medicine, Pusan National University Yangsan Hospital, 20 Geumo-ro, Mulgeum-eup, Yangsan 626-787, Korea
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Abstract
Understanding pancreatic development and the congenital anomalies and variants that result from alterations in normal development allows for better recognition of these anomalies at diagnostic imaging. This article reviews normal pancreatic embryology and anatomy, and the appearance of the more common developmental anomalies and ductal variants, with emphasis on computed tomography and magnetic resonance imaging. Common mimics of masses are also covered.
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Affiliation(s)
- Lauren F Alexander
- Department of Radiology, University of Alabama - Birmingham, 619 19th Street South, JTN355B, Birmingham, AL 35249-6830, USA.
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Yeom SK, Lee SW, Cha SH, Chung HH, Je BK, Kim BH, Hyun JJ. Biliary reflux detection in anomalous union of the pancreatico-biliary duct patients. World J Gastroenterol 2012; 18:952-9. [PMID: 22408355 PMCID: PMC3297055 DOI: 10.3748/wjg.v18.i9.952] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2011] [Revised: 11/08/2011] [Accepted: 01/18/2012] [Indexed: 02/06/2023] Open
Abstract
AIM: To demonstrate the imaging findings of biliopancreatic and pancreatico-biliary reflux in patients with anomalous union of the pancreatico-biliary duct (AUPBD) on gadoxetic acid-enhanced functional magnetic resonance cholangiography (fMRC).
METHODS: This study included six consecutive patients (two men and four women; mean age 47.5 years) with AUPBD. All subjects underwent endoscopic retrograde cholangiopancreatography (ERCP); one subject also underwent bile sampling of the common bile duct (CBD) to measure the amylase level because his gadoxetic acid-enhanced fMRC images showed evidence of pancreatico-biliary reflux of pancreatic secretions. Of the five patients with choledochal cysts, four underwent pylorus-preserving pancreaticoduodenectomy.
RESULTS: The five cases of choledochal cysts were classified as Todani classification I. In three of the six patients with AUPBD, injected contrast media reached the distal CBD and pancreatic duct on delay images, suggesting biliopancreatic reflux. In two of these six patients, a band-like filling defect was noted in the CBD on pre-fatty meal images, which decreased in size on delayed post-fatty meal images, suggesting pancreatico-biliary reflux of pancreatic secretions, and the bile sampled from the CBD in one patient had an amylase level of 113 000 IU/L. In one of the six patients with AUPBD, contrast media did not reach the distal CBD due to multiple CBD stones.
CONCLUSION: Gadoxetic acid-enhanced fMRC successfully demonstrated biliopancreatic reflux of bile and pancreatico-biliary reflux of pancreatic secretions in patients with AUPBD with and without choledochal cysts.
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Development and congenital anomalies of the pancreas. ANATOMY RESEARCH INTERNATIONAL 2011; 2011:351217. [PMID: 22567291 PMCID: PMC3335650 DOI: 10.1155/2011/351217] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/16/2010] [Accepted: 02/28/2011] [Indexed: 02/07/2023]
Abstract
Understanding how the pancreas develops is essential to understand the pathogenesis of congenital pancreatic anomalies. Recent studies have shown the advantages of investigating the development of frogs, mice, and chickens for understanding early embryonic development of the pancreas and congenital anomalies, such as choledochal cysts, anomalous pancreaticobiliary junction, annular pancreas, and pancreas divisum. These anomalies arise from failure of complete rotation and fusion during embryogenesis. There are many theories in the etiology of congenital anomalies of the pancreas. We review pancreas development in humans and other vertebrates. In addition, we attempt to clarify how developmental failure is related to congenital pancreatic anomalies.
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Ono S, Fumino S, Iwai N. Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today 2011; 41:601-5. [PMID: 21533929 DOI: 10.1007/s00595-010-4492-9] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2009] [Accepted: 11/14/2010] [Indexed: 02/07/2023]
Abstract
Pancreaticobiliary maljunction (PBM), which may be called by synonymous terms such as "anomalous arrangement of the pancreaticobiliary duct" or "anomalous union of biliopancreatic ducts," is defined as an anatomical maljunction of the pancreatic duct and the biliary duct outside of the duodenal wall beyond the influence of the sphincter of Oddi. Pancreaticobiliary maljunction is classified into three groups according to the type of the pancreaticobiliary junction. Pancreaticobiliary maljunction is diagnosed mainly by endoscopic retrograde cholangiopancreatography; however, in pediatric patients, it may be diagnosed by intraoperative cholangiography, and more recently has been diagnosed by computed tomographic cholangiography or contrast-enhanced helical computed tomography. Children with PBM without choledochal cysts usually do not show any symptoms except for abdominal pain and hyperamylasemia. Pancreaticobiliary maljunction is considered to be a major risk factor for biliary tract malignancy. In patients with PBM, free reflux of pancreatic juice into the biliary tract might induce biliary tract damage and biliary carcinogenesis. Thus, total resection of the extrahepatic bile duct and hepaticojejunostomy are recommended in children diagnosed with PBM with choledochal cyst. Early diagnosis and early surgical treatment provide a good prognosis with few complications. In addition, successive follow-up is necessary for early detection of biliary tract malignancy, especially in patients demonstrating postoperative complications.
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Affiliation(s)
- Shigeru Ono
- Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan
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Yang HH, He Y, Jin WX, Jin Y, Li HB, Jin CX. Progress in research of occult pancreatobiliary reflux. Shijie Huaren Xiaohua Zazhi 2010; 18:3886-3890. [DOI: 10.11569/wcjd.v18.i36.3886] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Increasing knowledge regarding pancreaticobiliary maljunction (PBM) has led researchers to find that pancreatobiliary reflux (flow of pancreatic juice into the biliary tract) occurs not only in PBM patients but also in individuals with a normal pancreaticobiliary junction (occult pancreatobiliary reflux, OPR). The functional disorders of the sphincter of Oddi have been proposed as a possible cause for OPR. Some studies have found that OPR is a high risk factor for gallbladder cancer. The incidence of choledochal stones is higher in patients with OPR than in those without OPR. In this article, we review the progress in research of OPR.
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Kosaka T, Tajima Y, Kuroki T, Mishima T, Adachi T, Tsuneoka N, Fukuda K, Kanematsu T. Helicobacter bilis colonization of the biliary system in patients with pancreaticobiliary maljunction. Br J Surg 2010; 97:544-9. [PMID: 20155791 DOI: 10.1002/bjs.6907] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Helicobacter bilis is considered to be a causative factor in the pathogenesis of biliary cancer. This study investigated the prevalence of H. bilis colonization of the biliary system of patients with pancreaticobiliary maljunction (PBM). METHODS Bile juice and biliary tissue samples were collected from 17 patients with PBM and 27 controls who had benign biliary disease without PBM. DNA extracted from each biliary sample was subjected to polymerase chain reaction (PCR) analysis for H. bilis and Helicobacter pylori. RESULTS PCR assays revealed that 12 of the 17 patients with PBM were positive for H. bilis DNA, compared with eight of 27 patients without PBM (P = 0.009). Among patients with PBM, H. bilis DNA was identified in six of eight children, including a 2-month-old infant, and in six of nine adults. The high prevalence of H. bilis DNA in the biliary system of patients with PBM was independent of age, sex, common bile duct dilatation, configuration of the pancreatic and bile ducts, and amylase activity in bile. CONCLUSION H. bilis colonization of the biliary system is extremely common in patients with PBM. This may point to a role in the pathogenesis of biliary cancer.
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Affiliation(s)
- T Kosaka
- Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8501, Japan.
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Pancreaticobiliary maljunction. Clin Gastroenterol Hepatol 2009; 7:S84-8. [PMID: 19896105 DOI: 10.1016/j.cgh.2009.08.024] [Citation(s) in RCA: 83] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2009] [Revised: 07/23/2009] [Accepted: 08/14/2009] [Indexed: 02/07/2023]
Abstract
Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. In PBM patients, this anomaly allows regurgitation between the pancreatobiliary and biliopancreatic tract. Since hydrostatic pressure within the pancreatic duct is usually higher than that in the common bile duct, pancreatic juice frequently refluxes into the bile duct. As a result, pancreatic enzyme levels are generally very high in the bile and there is a related high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst [CCC]) and PBM without biliary dilatation (maximal diameter of the bile duct <or= 10 mm). In 42 CCC patients, bile duct and gallbladder cancer was seen in 7 and in 8, respectively. In 49 PBM patients without biliary dilatation, only gallbladder cancer was detected in 33 patients. Because of the increased cell proliferation with random K-ras mutations, the gallbladder mucosa of PBM patients should be considered premalignant. CCC treatment is prophylactic flow-diversion surgery, but only prophylactic cholecystectomy is usually performed for PBM without biliary dilatation. Pancreatobiliary reflux and premalignant changes in the gallbladder can occur in patients with a relatively long common channel (high confluence of pancreaticobiliary ducts).
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The changing presentation of choledochal cyst disease: an incidental diagnosis. HPB SURGERY : A WORLD JOURNAL OF HEPATIC, PANCREATIC AND BILIARY SURGERY 2009; 2009:103739. [PMID: 19841688 PMCID: PMC2763265 DOI: 10.1155/2009/103739] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 03/19/2009] [Revised: 05/27/2009] [Accepted: 08/24/2009] [Indexed: 02/07/2023]
Abstract
BACKGROUND Choledochal cysts are uncommon biliary lesions. Due to the evolution of imaging and laparoscopic surgery, we sought to describe our last 3 years experience with the presentation and management of choledochal cysts in adults. METHODS A retrospective review of a prospectively established database of adults who were managed for primary choledochal cyst disease between 2005 and 2008 was performed. RESULTS Between 8/2005 and 8/2008, 14 adults were managed for primary choledochal cyst disease. The average age was 41 years (range 17-86) and 79% were female. Presentations included biliary sepsis (3), pancreatitis (2), abdominal pain (3), or painless jaundice (1). Three patients had the cyst found during laparoscopic cholecystectomy, and two had an incidental finding after CT scan for an unrelated issue. The length of stay for those who had the cyst removed was 7.8 days (range 5-11). There were no operative or post-operative complications. CONCLUSIONS Over the last 3 years 36% of our patients with choledochal cysts presented after incidental finding, either during a laparoscopic operation or after a CT scan for an unrelated problem. Increasing utilization of laparoscopy and CT scan for abdominal complaints has lead to a change in the pattern of presentation.
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Arakura N, Ozaki Y, Muraki T, Maruyama M, Chou Y, Kodama R, Takayama M, Hamano H, Tanaka E, Kawa S. Pancreaticobiliary fistula associated with pancreatolithiasis. Clin J Gastroenterol 2009; 2:226-231. [DOI: 10.1007/s12328-009-0070-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2009] [Accepted: 02/16/2009] [Indexed: 11/30/2022]
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p53 gene mutation and p53 protein overexpression in a patient with simultaneous double cancer of the gallbladder and bile duct associated with pancreaticobiliary maljunction. ACTA ACUST UNITED AC 2009; 16:376-81. [PMID: 19183832 DOI: 10.1007/s00534-008-0030-1] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2007] [Accepted: 01/31/2008] [Indexed: 10/21/2022]
Abstract
Pancreaticobiliary maljunction (PBM) is associated with the occurrence of biliary cancer due to pancreatobiliary reflux. We present a case of simultaneous double cancer of the gallbladder and bile duct. A 77-year-old woman who had jaundice, intra- and extra-hepatic biliary ductal dilatation and a space-occupying lesion in the gallbladder and lower bile duct underwent pancreatoduodenectomy. The gallbladder cancer showed papillary carcinoma without mutation of the K-ras gene and with p53 non-sense mutation of CCA (Pro) to CA (Stop) on codon 301 in exon 8. The bile duct cancer revealed a well-differentiated adenocarcinoma without mutation of the K-ras gene and with p53 miss-sense mutation of GTG (Val) to GAG (Glu) on codon 272 in exon 8. There were no mutations of either the K-ras or p53 gene in non-cancerous epithelia. In contrast, only the mucosa of the common channel had p53 protein accumulation and high cell proliferation activity. Therefore, the genetic pathway might be the same in both the gallbladder and bile duct cancer, and a high potential for carcinogenesis might be present in the epithelium of the common channel in patients with PBM.
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Tajima Y, Kitajima T, Tomioka T, Eto T, Inoue K, Fukahori T, Sasaki M, Tsunoda T. Hamster Models of Biliary Carcinoma. HEPATOBILIARY AND PANCREATIC CARCINOGENESIS IN THE HAMSTER 2009:29-68. [DOI: 10.1007/978-4-431-87773-8_5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Kamisawa T, Anjiki H, Egawa N, Kurata M, Honda G, Tsuruta K. Diagnosis and clinical implications of pancreatobiliary reflux. World J Gastroenterol 2008; 14:6622-6. [PMID: 19034962 PMCID: PMC2773301 DOI: 10.3748/wjg.14.6622] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
The sphincter of Oddi is located at the distal end of the pancreatic and bile ducts and regulates the outflow of bile and pancreatic juice. A common channel can be so long that the junction of the pancreatic and bile ducts is located outside of the duodenal wall, as occurs in pancreaticobiliary maljunction (PBM); in such cases, sphincter action does not functionally affect the junction. As the hydropressure within the pancreatic duct is usually greater than in the bile duct, pancreatic juice frequently refluxes into the biliary duct (pancreatobiliary reflux) in PBM, resulting in carcinogenetic conditions in the biliary tract. Pancreatobiliary reflux can be diagnosed from elevated amylase level in the bile, secretin-stimulated dynamic magnetic resonance cholangiopancreatography, and pancreatography via the minor duodenal papilla. Recently, it has become obvious that pancreatobiliary reflux can occur in individuals without PBM. Pancreatobiliary reflux might be related to biliary carcinogenesis even in some individuals without PBM. Since few systemic studies exist with respect to clinical relevance and implications of the pancreatobiliary reflux in individuals with normal pancreaticobiliary junction, further prospective clinical studies including appropriate management should be performed.
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36
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Nakamura H, Katayose Y, Rikiyama T, Onogawa T, Yamamoto K, Yoshida H, Hayashi H, Ohtsuka H, Hayashi Y, Egawa SI, Unno M. Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease. ACTA ACUST UNITED AC 2008; 15:554-9. [DOI: 10.1007/s00534-007-1310-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2007] [Accepted: 09/21/2007] [Indexed: 02/07/2023]
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Kogure H, Tsujino T, Yagioka H, Sasaki T, Nakai Y, Hirano K, Sasahira N, Isayama H, Tada M, Kawabe T, Omata M. Self-expandable metallic stents for malignant biliary obstruction with an anomalous pancreaticobiliary junction. Surg Endosc 2008; 22:787-91. [PMID: 17704880 DOI: 10.1007/s00464-007-9482-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
BACKGROUND Anomalous pancreaticobiliary junction (APBJ) is associated with pancreaticobiliary cancer. Limited data are available on endoscopic biliary drainage for unresectable malignant biliary obstruction with APBJ. This study evaluated the efficacy and safety of self-expandable metallic stents (EMSs) for the management of malignant biliary obstruction with APBJ. METHODS Between 1993 and 2005, 324 patients with unresectable malignant biliary obstruction underwent insertion of an EMS. Six of these patients with concomitant APBJ constituted the subjects of this study. Early (</=30 days after EMS insertion) and late (>30 days after EMS insertion) stent-related complications and stent patency were evaluated in these six patients. RESULTS The cause of biliary obstruction was gallbladder cancer in four patients and pancreatic cancer in two patients. Uncovered EMSs were inserted across the common channel without performance of a biliary sphincterotomy. The diameter of the uncovered EMS used was based on the diameter of the common channel. For all six patients, endoscopic biliary drainage was successful, and their jaundice subsided steadily. None of the six patients experienced early complications, including acute pancreatitis. The mean stent-related complication-free period was 163 days. Stent occlusion caused by tumor ingrowth occurred in two patients. Acute cholangitis and cholecystitis were observed in one patient each. CONCLUSIONS Uncovered EMSs are effective for palliation of unresectable malignant biliary obstruction in patients who have APBJ without increasing the risk of stent-related early complications.
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Affiliation(s)
- H Kogure
- Department of Gastroenterology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
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Kianmanesh R, Scaringi S, Castel B, Flamant Y, Msika S. [Precancerous lesions of the gallbladder]. ACTA ACUST UNITED AC 2008; 144:278-86. [PMID: 17925730 DOI: 10.1016/s0021-7697(07)91953-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
This Mini-review summarizes the epidemiology, predisposing and pre-cancerous conditions related to carcinoma of the gallbladder. In 75% of cases, gallbladder cancer is a cholangiocarcinoma, usually presenting in a late and advanced stage, and it carries one of the worst prognoses of all GI malignancies. Early stage disease is usually discovered incidentally by the pathologist in a gallbladder specimen removed for calculous cholecystitis. It occurs three times more frequently in women than in men and invasive forms usually occur after the age of 60. Incidence varies with geographic location. Besides genetic and geographic factors, the presence of one or more large gallstones is a major risk factor. Gallbladder polyps larger than 1.5 cm. (especially solitary sessile hypoechogenic polyps) are associated with a 50% risk of malignancy. Choledochal cysts and other variations of the biliopancreatic junction are also associated with high risk; cancer may occur at a much younger age in these patients and in the absence of gallstones. Porcelain gallbladder is a risk factor, particularly when there is calcification of the gallbladder mucosa. Chronic gallbladder infection has been implicated as a risk factor for malignant degeneration. Finally, cancer of both the gallbladder and the bile ducts is more frequent in patients suffering from primary biliary cirrhosis.
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Affiliation(s)
- R Kianmanesh
- Service de Chirurgie Générale et Digestive, Hôpital Louis Mourier AP-HP, Université Paris VII - Colombes.
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Abstract
Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins, autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted.
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Kamisawa T, Tu Y, Nakajima H, Egawa N, Tsuruta K, Okamoto A, Matsukawa M. Acute pancreatitis and a long common channel. ACTA ACUST UNITED AC 2007; 32:365-9. [PMID: 16955378 DOI: 10.1007/s00261-006-9057-4] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
BACKGROUND As sphincter action does not functionally affect the union in pancreaticobiliary maljunction (PBM), two-way regurgitation occurs. We investigated the features of acute pancreatitis associated with patients displaying a long common channel. METHODS We reviewed 3210 endoscopic retrograde cholangiopancreatograms. PBM was diagnosed in 107 patients with a long common channel in which communication between the pancreatic and bile duct was maintained even during sphincter contraction. High confluence of pancreaticobiliary ducts (HCPBD) was diagnosed in 60 patients with a common channel >or=6 mm long in which communication between the two ducts was occluded with sphincter contraction. RESULTS Of patients with PBM, four had acute pancreatitis. Etiology of acute pancreatitis in the two patients was suspected to involve impaction in a long common channel. Of patients with HCPBD, 17 displayed acute pancreatitis. Three patients had a history of excessive alcohol intake and 10 patients were associated with choledocholithiasis or cholecystitis. The remaining four patients had no history of them. CONCLUSIONS Acute pancreatitis occurs in 13% of patients with a long common channel. Obstruction of a long common channel by stones easily induces bile flow into the pancreas. Even if no obstruction is present, biliopancreatic reflux induces acute pancreatitis in some patients.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Tokyo, 113-8677, Japan.
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Motosugi U, Ichikawa T, Araki T, Kitahara F, Sato T, Itakura J, Fujii H. Secretin-stimulating MRCP in patients with pancreatobiliary maljunction and occult pancreatobiliary reflux: direct demonstration of pancreatobiliary reflux. Eur Radiol 2007; 17:2262-7. [PMID: 17447071 DOI: 10.1007/s00330-007-0640-z] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2006] [Revised: 02/28/2007] [Accepted: 03/22/2007] [Indexed: 12/22/2022]
Abstract
We propose the hypothesis that the enlargement of the common bile duct (CBD) or gallbladder (GB) that is occasionally demonstrated on magnetic resonance cholangiopancreatography (MRCP) after secretin stimulation is caused by pancreatobiliary reflux. Recently, occult pancreatobiliary reflux (OPR) has been demonstrated in patients without morphological pancreatobiliary maljunction (MPBM). The aim of this study was to evaluate the efficacy of secretin-stimulating MRCP (SMRCP) in the diagnosis of pancreatobiliary reflux. The study included 14 patients with MPBM and 32 patients with a normal pancreatobiliary junction. OPR was evaluated by bile collection and diagnosed in seven of the 32 patients. All the patients underwent SMRCP; the related findings were considered positive when enlargement of the CBD or GB was observed. Positive findings on SMRCP were observed in all MPBM patients. In the patients with normal pancreatobiliary junction, there was significant difference between the mean amylase levels in the patients with positive and negative SMRCP findings (mean, 4,755.7 and 29.7 IU/l). The sensitivity and specificity of SMRCP for diagnosing OPR was 85.7% and 68.0%, respectively. SMRCP provides a non invasive method for excluding PBR and can identify patients who could benefit from bile duct sampling to diagnose OPR.
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Affiliation(s)
- Utaroh Motosugi
- Department of Radiology, University of Yamanashi, 1110 Shimokato, Chuo-shi, Yamanashi, 409-3898, Japan.
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Cano DA, Hebrok M, Zenker M. Pancreatic development and disease. Gastroenterology 2007; 132:745-62. [PMID: 17258745 DOI: 10.1053/j.gastro.2006.12.054] [Citation(s) in RCA: 72] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2006] [Accepted: 12/20/2006] [Indexed: 12/22/2022]
Affiliation(s)
- David A Cano
- Diabetes Center, Department of Medicine, University of California San Francisco, 94143, USA
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Søreide K, Søreide JA. Bile duct cyst as precursor to biliary tract cancer. Ann Surg Oncol 2006; 14:1200-11. [PMID: 17187167 DOI: 10.1245/s10434-006-9294-3] [Citation(s) in RCA: 62] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2006] [Revised: 09/24/2006] [Accepted: 09/25/2006] [Indexed: 02/07/2023]
Abstract
BACKGROUND Bile duct cysts (BDC) are rare, of uncertain origin, and occur most often in young females of Asian descent. Increasingly, BDCs are reported in the Western population, often with coexistent biliary tract cancer. METHODS The PubMed and Medline literature databases were searched for pertinent publications regarding the clinical association and molecular biological development of cancerogenesis in BDC. Reports from the last two decades were emphasized. RESULTS Cancer is found in 10-30% of adults with BDC. The cancer-risk is low in childhood (<1% in the first decade), and shows a clear increase with age. Cholangiocarcinoma is the most common malignancy in BDC, and represents a 20- to 30-fold risk compared to the general population. The mean age of malignancy in BDC is 32 years (about two decades earlier than in the general population). Type I and type IV cysts show a higher cancer incidence, even after cyst excision. Pathological findings strongly suggest a hyperplasia-dysplasia-carcinoma sequence in carcinogenesis of pancreatico-biliary maljunction (PBM). Reflux of pancreatic enzymes, amylase, bile stasis, and an increased intraductal concentration of bile acids contribute to proliferative activity of bile acids in BDC. While microsatellite instability, k-ras mutations, expression of COX-2 and bcl-2, and increased telomerase activity seem to occur early; involvement of cyclin D1, beta-catenin, DPC-4/Smad4 and p53 appear later in carcinogenesis. CONCLUSION Increased molecular knowledge substantiates the clinically related cancer-risk in BDC. Surgery remains the golden standard for treatment, relieves patients from associated complications, and interrupts the cancerous potential in BDC.
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Affiliation(s)
- Kjetil Søreide
- Department of General and Gastroenterologic Surgery, Stavanger University Hospital, Stavanger, Norway.
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Franko J, Nussbaum ML, Morris JB. Choledochal cyst cholangiocarcinoma arising from adenoma: case report and a review of the literature. ACTA ACUST UNITED AC 2006; 63:281-4. [PMID: 16843781 DOI: 10.1016/j.cursur.2006.04.010] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2006] [Revised: 04/05/2006] [Accepted: 04/18/2006] [Indexed: 02/07/2023]
Abstract
A case of cholangiocarcinoma arising in an unresected choledochal cyst in adulthood is presented. Although typically diagnosed in pediatric population, as many as 20% to 30% of choledochal cysts can be discovered in adulthood. Unresected choledochal cyst is clearly associated with increased risk of cholangiocarcinoma. Proper surgical treatment includes cyst resection and bilioenteric anastomosis. Asymptomatic patients with choledochal cyst previously treated by biliary diversion without cyst resection present a challenging issue and should be considered for cyst resection. Association of choledochal cyst with pancreaticobiliary anomalies is reviewed.
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Affiliation(s)
- Jan Franko
- Department of Surgery, Abington Memorial Hospital, PA 19001, USA.
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Takeda K, Sekido H, Sugita M, Tanaka K, Endo I, Togo S, Shimada H. Porcelain gallbladder complicated with pancreas divisum. ACTA ACUST UNITED AC 2006; 13:580-3. [PMID: 17139436 DOI: 10.1007/s00534-006-1116-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2006] [Accepted: 02/14/2006] [Indexed: 10/23/2022]
Abstract
We report a rare case of porcelain gallbladder associated with pancreas divisum (PD). A 60-year-old woman suffered from discomfort in the back of the right side. An abdominal radiograph revealed a calcified spherical mass in the right upper quadrant. Ultrasonography revealed a scattered echo with a posterior acoustic shadow in the gallbladder wall. A plain computed tomography (CT) scan showed flecks of intramural calcification in the wall of the gallbladder. Endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) showed separate openings for the Santorini and Wirsung ducts. The patient underwent cholecystectomy after porcelain gallbladder and pancreas divisum had been diagnosed. The porcelain gallbladder resulted from a stone impacted in the neck of the gallbladder. Patients with PD should be followed carefully, because gallstones often accompany PD, and porcelain gallbladder may result, as in this patient.
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Affiliation(s)
- Kazuhisa Takeda
- Department of Gastroenterological Surgery, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan
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Schaefer JF, Kirschner HJ, Lichy M, Schlemmer HP, Schick F, Claussen CD, Fuchs J. Highly resolved free-breathing magnetic resonance cholangiopancreatography in the diagnostic workup of pancreaticobiliary diseases in infants and young children--initial experiences. J Pediatr Surg 2006; 41:1645-51. [PMID: 17011262 DOI: 10.1016/j.jpedsurg.2006.05.052] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
PURPOSE A new generation of magnetic resonance imaging scanner offers a 3-dimensional magnetic resonance cholangiopancreatography (MRCP) with very high spatial resolution using a non-breath-hold technique with prospective navigator gating. The study aimed to investigate the new technology of the MRCP in infants with suspected pancreaticobiliary diseases. METHODS Seven patients (4 girls, 3 boys; mean age, 2.8 years; range, 3 months to 5.6 years) were examined. All patients underwent magnetic resonance imaging examination using a 1.5-T whole-body scanner (Magnetom Avanto, Siemens Medical, Erlangen, Germany). For MRCP, a heavily T2-weighted (time to echo, 678 ms) 3-dimensional turbo spin echo in coronal plane was performed with the voxel size of 1 x 1 x 1.5 mm. To enable non-breath-holding imaging, a diaphragm navigator sequence was simultaneously carried out. The MRCP findings were compared prospectively with the endoscopic retrograde cholangiopancreatography and/or with the surgical findings in 6 of 7 cases. In 1 patient, the follow-up was considered reference standard. RESULTS The high spatial resolution of the images without breathing artifacts allowed an excellent demonstration of all relevant pancreaticobiliary ducts and of the pathologic findings. All patients (n = 4) with choledocholithiasis were identified through MRCP. The finding of a high pancreaticobiliary junction (n = 3) was confirmed by endoscopic retrograde cholangiopancreatography in all cases. The length of the common channel ranged from 16 to 24 mm. In 1 patient, the long common channel was associated with a choledochal cyst. In a 1.7-year-old boy with recurrent abdominal pain and mild pancreatitis, the rare case of a gastric duplication with connection to the main pancreatic duct was diagnosed. A regular gastric wall with normal mucosa and parts of the duodenal wall with a circular layer of muscle (pylorus-like) were found in histology after surgical resection of the mass. CONCLUSION The newly developed technique may be a reliable tool in the workup of pancreaticobiliary diseases in infants.
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Affiliation(s)
- Juergen F Schaefer
- Department of Diagnostic Radiology, University of Tuebingen, 72076 Tuebingen, Germany.
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Adachi T, Tajima Y, Kuroki T, Mishima T, Kitasato A, Fukuda K, Tsutsumi R, Kanematsu T. Bile-reflux into the pancreatic ducts is associated with the development of intraductal papillary carcinoma in hamsters. J Surg Res 2006; 136:106-11. [PMID: 16863651 DOI: 10.1016/j.jss.2006.04.025] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2006] [Revised: 04/07/2006] [Accepted: 04/24/2006] [Indexed: 01/12/2023]
Abstract
BACKGROUND Reflux of pancreatic juice into the biliary tract is a well-known risk factor for the development of biliary carcinoma. In this study, we investigated the significance of bile-reflux into the pancreatic ducts in pancreatic carcinogenesis, especially in the development of carcinoma in the main pancreatic duct in hamsters. MATERIALS AND METHODS Syrian hamsters were subjected to three different surgical procedures: cholecystoduodenostomy with dissection of the extrahepatic bile duct on the distal end of the common duct (Model A); cholecystoduodenostomy along with a dissection of the common bile duct (Model B); or simple laparotomy (Model C). The animals then received weekly subcutaneous injections of N-nitrosobis(2-oxopropyl)amine (BOP), for 9 weeks, and were killed for pathological investigation at 16 weeks after the initial BOP administration. RESULTS Pancreas carcinomas developed in 95, 88, and 90% of the Model A hamsters (n = 22), B (n = 24), and C (n = 21), respectively. The induced pancreatic tumors were histologically classified into four types: papillary; tubular; cystic adenocarcinoma; or intraductal carcinoma of the main pancreatic duct consisting of intraductal papillary carcinoma (IPC) and intraductal tubular carcinoma (ITC). The number and the incidence of IPCs induced in Model A hamsters were 24 lesions and 77% and were statistically higher than those in Model B (7 lesions and 29%) and C hamsters (7 lesions and 33%) (P < 0.01). Bile-reflux into the pancreatic ducts was clearly demonstrated in only hamsters of Model A by means of Indocyanine green injection via the portal vein. Proliferative cell nuclear antigen labeling indices of the epithelial cells in the main pancreatic duct in hamsters, with no BOP treatment, were 3.8, 0.8, and 1.1% in Models A (n = 10), B (n = 10), and C (n = 10), respectively, and the difference was statistically significant (P < 0.01). CONCLUSIONS Our findings suggest that bile-reflux into the pancreatic ducts is a significant factor predisposing to the development of IPC of the pancreas through an acceleration of epithelial cell kinetics of the main pancreatic duct.
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Affiliation(s)
- Tomohiko Adachi
- Department of Transplantation and Digestive Surgery, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan.
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Kamisawa T, Kuwata G, Yui CP, Tu Y, Fujiwara T, Endoh J, Arakawa T, Koizumi K, Nakajima H, Egawa N. LESIONS WITH A HIGH RISK OF CARCINOGENESIS IN THE GALLBLADDER OF PATIENTS WITH A LONG COMMON CHANNEL. Dig Endosc 2006. [DOI: 10.1111/j.0915-5635.2006.00603.x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/23/2023]
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Abstract
BACKGROUND Gallbladder cancer is an uncommon disease except in countries like Chile and areas of India and Japan. The knowledge regarding the etiology and mechanisms through which this neoplasia is developed is significantly less compared to other malignant tumors. RESULTS The epithelial lesions involved in gallbladder carcinogenesis are dysplasia and adenomas that represent two biologically distinct carcinogenetic models. Dysplasia progresses to carcinoma in situ (CIS) and subsequently becomes invasive. Over 80% of invasive gallbladder cancers present areas adjacent to the CIS and epithelial dysplasia. Other authors have demonstrated adenomatous areas in carcinomas, or malignant transformation in an adenoma. The low incidence of gallbladder adenomas (0.14% of cholecystectomies) and the presence of adenomatous remnants in the neighboring mucosa to early carcinomas in less than 3% of the cases suggest the limited importance of this carcinogenic pathway. Epithelial dysplasia which is not associated with gallbladder cancer is observed in approximately 1% of cholecystectomies for symptomatic lithiasis. Metaplasia, dysplasia, and CIS are present in the mucosa adjacent to the cancer in 66%, 81.3%, and 69%, respectively. The average ages of patients with dysplasia not associated to cancer (51.9 years), early carcinomas (56.8 years), and advanced carcinomas (62.9 years) demonstrate a gradient which suggests the progression of these lesions. CONCLUSIONS From the morphological point of view, the dysplasia-carcinoma sequence is the most plausible carcinogenic pathway for gallbladder cancer, a process which would require a period of approximately 10 years.
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Affiliation(s)
- Iván Roa
- Department of Pathology, Faculty of Medicine, Universidad de la Frontera, Temuco, Chile
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Oyachi N, Ohhama Y, Take H, Fukuzato Y, Murakami T, Kitagawa N, Kudo H. Aplasia of the dorsal pancreas and choledochal cyst. Pediatr Surg Int 2006; 22:557-9. [PMID: 16474976 DOI: 10.1007/s00383-006-1652-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/23/2006] [Indexed: 11/30/2022]
Abstract
The authors describe a rare case of choledochal cyst and aplasia of the dorsal pancreas complicated with chronic pancreatitis. A 9-year-old boy presented with obstructive jaundice. After biliary drainage using pericutaneous transhepatic gallbladder drainage (PTGBD) technique, the patient underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. The association of choledochal cyst and aplasia of the dorsal pancreas with chronic pancreatitis has never been reported previously.
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Affiliation(s)
- Noboru Oyachi
- Department of Surgery, Kanagawa Children's Medical Center, 2-138-4 Mutsukawa, Minami-ku, 232-8555, Yokohama, Kanagawa, Japan.
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