Pancreaticobiliary Maljunction: Markedly High Risk for Biliary Cancer

Kamisawa, Terumi; Honda, Goro

doi:10.1159/000490816

Cited by 16 publications

(16 citation statements)

References 7 publications

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“…Another hypothesis suggests gallbladder carcinomas and MiNEN may arise consequential to the classical adenoma-carcinoma sequence [ 1 , 15 ]. It is well documented that gastric and intestinal metaplasia changes with neuroendocrine cells occur secondary to chronic cholecystitis [ 1 , 10 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction

Fick¹,

Tran²,

Kandpal³

et al. 2023

Cureus

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Gallbladder neuroendocrine tumors (GB-NETs) and gallbladder neuroendocrine carcinomas (GB-NECs) are rare forms of neuroendocrine neoplasms (NENs). Most GB-NENs present as incidental findings or as gallbladder polyps in the course of investigation of nonspecific symptoms such as upper abdominal pain and nausea. Given the rarity of GB-NENs, only a few small case series are currently available describing this entity, and even fewer guiding consensus on standard treatment and the role of adjuvant therapy. We present the case of a 65-year-old South American female referred for a chronic history of intermittent post-prandial epigastric pain, bloating, nausea, and occasional diarrhea. Pancreaticobiliary maljunction with dilation was present and she was diagnosed with primary gallbladder large cell neuroendocrine carcinoma (GB-LCNEC) mixed with a minor component of gallbladder adenocarcinoma.

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Section: Discussionmentioning

confidence: 99%

An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction

Fick¹,

Tran²,

Kandpal³

et al. 2023

Cureus

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“…PBM, also known as abnormal or anomalous junction/union of the pancreatobiliary ductal system, is a congenital malformation with a markedly high risk for biliary cancer. In PBM patients with biliary tract cancers, bile duct and gallbladder cancers develop at a mean age of 50–60 years, which is approximately 15–20 years earlier compared to those without PBM [ 48 , 49 , 50 , 51 ]. PBM is defined as a union of the pancreatic and biliary ducts located outside of the duodenal wall, where they merge in the supra-Oddi region.…”

Section: Pancreatobiliary Maljunctionmentioning

confidence: 99%

“…For PBM cases with CBD or with spindle/cylindrical type of dilatation without cancer, excision of the extrahepatic biliary tract with biliary reconstruction is the treatment of choice nowadays. On the contrary, treatment of PBM without an association with biliary dilatation and without cancer is controversial, and there is no fixed viewpoint at present [ 48 , 49 ]. Some surgeons insist that only cholecystectomy should be performed and excision of extrahepatic biliary tract in unnecessary because of the very high incidence of gallbladder cancer and low risk of bile duct carcinogenesis.…”

Section: Pancreatobiliary Maljunctionmentioning

confidence: 99%

Precursor Lesions of Gallbladder Carcinoma: Disease Concept, Pathology, and Genetics

et al. 2022

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Understanding the pathogenesis and carcinogenesis of gallbladder adenocarcinoma is important. The fifth edition of the World Health Organization’s tumor classification of the digestive system indicates three types of preinvasive neoplasm of the gallbladder: pyloric gland adenoma (PGA), biliary intraepithelial neoplasia (BilIN), and intracholecystic papillary neoplasm (ICPN). New terminologies have also been introduced, such as intracholecystic papillary-tubular neoplasm, gastric pyloric, simple mucinous type, and intracholecystic tubular non-mucinous neoplasm (ICTN). Pancreatobiliary maljunction (PBM) poses a markedly high risk for bile duct carcinoma, which was analyzed and investigated mainly by Asian researchers in the past; however, recent studies have clarified a similar significance of biliary carcinogenesis in Western countries as well. In this study, we reviewed and summarized information on three gallbladder neoplastic precursors, PGA, BilIN, and ICPN, and gallbladder lesions in patients with PBM.

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“…The main anatomical feature of PBM is that the bile duct and pancreatic duct join out of the duodenal wall, forming a lengthy common duct (Figure 1 )[ 2 , 3 ], often combined with sphincter of Oddi dysplasia. As a result, the pancreatic duct and bile duct lose control, causing reflux[ 4 ]. Due to this anatomical abnormality, PBM is often associated with certain diseases, such as cholelithiasis, cholangitis, pancreatitis, and increased risk of cholangiocarcinoma[ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Application of imaging techniques in pancreaticobiliary maljunction

Wang¹,

Mu²,

Xu³

et al. 2022

WJCC

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Imaging techniques are useful tools in the diagnosis and treatment of pancreaticobiliary maljunction (PBM). PBM is a precancerous lesion often relative to the disease of the pancreas and biliary tract, for example, cholecystolithiasis, protein plugs, and pancreatitis. For patients with PBM, early diagnosis and timely treatment are highly important, which is largely dependent on imaging techniques. The continuous development of imaging techniques, including endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, computed tomography, ultrasound, and intraoperative cholangiography, has provided appropriate diagnostic and therapeutic tools for PBM. Imaging techniques, including non-invasive and invasive, have distinct advantages and disadvantages. The purpose of this paper is to review the application of various imaging techniques in the diagnosis and treatment of PBM.

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Pancreaticobiliary Maljunction: Markedly High Risk for Biliary Cancer

Cited by 16 publications

References 7 publications

An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction

An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction

Precursor Lesions of Gallbladder Carcinoma: Disease Concept, Pathology, and Genetics

Application of imaging techniques in pancreaticobiliary maljunction

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