A case of congenital choledochal cystassociated with carcinoma

Fujiwara, Yoshiyuki; Ohizumi, Tetsunosuke; Kakizaki, G; Ishidate, Takuzo

doi:10.1016/s0022-3468(76)80021-8

Cited by 24 publications

(11 citation statements)

References 2 publications

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“…In the published literature, all pediatric patients who developed biliary tract cancer were reported to have PBM. [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] This supports the hypothesis that epithelial changes of the gallbladder, leading to carcinogenesis, may occur in early childhood in these patients. We also speculate that patients with PBM have the possibility of producing precancerous lesions of the biliary tree early in childhood.…”

Section: Biliary Tract Malignancy With Pbmsupporting

confidence: 55%

“…22 In a review of the literature, several investigators were found to have reported cholangiocarcinoma that developed in patients younger than 20 years old who had PBM and choledochal cysts. [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] In the presence of PBM, free refl ux of pancreatic juice into the biliary tract can occur, and this may damage the biliary epithelium. In fact, epithelial hyperplasia of the biliary tract was frequently observed in patients with choledochal cysts with PBM.…”

Section: Biliary Tract Malignancy With Pbmmentioning

confidence: 99%

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Diagnosis and treatment of pancreaticobiliary maljunction in children

2011

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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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Section: Biliary Tract Malignancy With Pbmsupporting

confidence: 55%

Section: Biliary Tract Malignancy With Pbmmentioning

confidence: 99%

Diagnosis and treatment of pancreaticobiliary maljunction in children

2011

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“…There have been previous reports of cholangiocarcinoma developing in children with CC with AAPBD [3][4][5][6][7][8][9], and biliary epithelial hyperplasia has been observed in some [2]. This hyperplastic change seems to be triggered by chronic inflammation caused by the reflux of pancreatic juice leading perhaps to metaplasia [16].…”

Section: Discussionmentioning

confidence: 99%

“…In 1969, Babbitt [1] reported an AAPBD in 3 children with CCs, in which he proposed that is was the cause of the dilatation. The combination is widely held to be a risk factor for biliary tract malignancy [2], and several investigators have reported cholangiocarcinoma developing in patients younger than 20 years [3][4][5][6][7][8][9] and others have shown carcinoma arising from the intrahepatic bile duct or residual common bile duct following resection of their CC [10,11]. Nonetheless, most patients with CC have a good prognosis when diagnosed and treated early.…”

mentioning

confidence: 99%

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

Ono

Fumino

Shimadera

et al. 2010

Journal of Pediatric Surgery

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“…The younger the patient at presentation of the choledochal cyst, the lower the incidence of subsequent malignant change: the risk is less than 1% if the choledochal cyst presents within the first decade of life, but increases to 14% if presentation is delayed beyond 20 years of age 12. The youngest reported patient with adenocarcinoma in a choledochal cyst was 17 years old, and she had undergone biliary bypass 8 years earlier 13…”

Section: Malignant Changementioning

confidence: 99%

Biliary cystic disease: the risk of cancer

Benjamin

2003

Journal of Hepato-Biliary-Pancreatic Surgery

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Congenital choledochal cysts carry a risk of cancer, probably as a result of a sequence of pancreatobiliary reflux, inflammation, dysplasia with or without intestinal metaplasia, and invasive carcinoma. A combination of biliary stasis due to poor drainage of a stagnant pool of bile and increased mutagenicity of the bile acids may be ultimately responsible. There is very frequently an anomalous pancreato-biliary ductal junction, and the reflux of pancreatic juice into the bile duct is thought to play a central role in the process of carcinogenesis. The risk of cancer is low in childhood (under 1% in the first decade), but shows a clear increase with age (over 10% in the third decade). The implication for management is that total excision of the extrahepatic biliary tree at risk remains the gold standard for management of these cysts, and simple bypass in infancy or childhood leaves the risk of cancer, though possibly diminished, still significant.

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A case of congenital choledochal cystassociated with carcinoma

Cited by 24 publications

References 2 publications

Diagnosis and treatment of pancreaticobiliary maljunction in children

Diagnosis and treatment of pancreaticobiliary maljunction in children

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

Biliary cystic disease: the risk of cancer

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