1972
DOI: 10.1136/jcp.25.9.804
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Congenital dilatation of the intrahepatic bile ducts with cholangiocarcinoma

Abstract: Intrahepatic cholangiocarcinomas were found at necropsy in two previously reported cases of congenital dilatation of the intrahepatic bile ducts. The nature of the developmental abnormality is discussed and compared with other forms of biliary dilatation. Slow-flowing bile for many years probably leads to cholangiocarcinoma.

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Cited by 108 publications
(30 citation statements)
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“…ICC is also known to occur in thorotrast deposition, liver flukes (Clonorchis sinesis and Opisthorchis viverrini) infestations, and congenital biliary anomalies. 1,14,[25][26][27][28][29] In liver fluke infestation, the bile ducts first show desquamation of the biliary epithelium with subsequent development of adenomatous hyperplasia and periductal fibrosis in addition to inflammation and goblet cell metaplasia. 30 Neoplastic transformation from adenomatous change in bile ducts to ICC through dysplastic changes is also demonstrable in Opisthorchiasis.…”
Section: Discussionmentioning
confidence: 99%
“…ICC is also known to occur in thorotrast deposition, liver flukes (Clonorchis sinesis and Opisthorchis viverrini) infestations, and congenital biliary anomalies. 1,14,[25][26][27][28][29] In liver fluke infestation, the bile ducts first show desquamation of the biliary epithelium with subsequent development of adenomatous hyperplasia and periductal fibrosis in addition to inflammation and goblet cell metaplasia. 30 Neoplastic transformation from adenomatous change in bile ducts to ICC through dysplastic changes is also demonstrable in Opisthorchiasis.…”
Section: Discussionmentioning
confidence: 99%
“…A MEDLINE search of all pertinent articles yielded reports of 12 patients in whom bile duct cancer developed after the primary resection of congenital biliary dilation (Table 1). [1][2][3][4][5][6][7][8][9] The 13 patients, including our own, consisted of 3 men and 10 women, and they ranged in age from 16 to 61 years. The mean period between the primary operation and the development of cancer was 8.2 years, ranging from 1.8 to 19.5.…”
Section: Discussionmentioning
confidence: 99%
“…However, some patients have been reported to develop biliary cancer long after a "separation-operation". [1][2][3][4][5][6][7][8][9] Most of the development in these patients occurs in the dilated bile duct (for instance, in the remnant choledochal cyst) in the hepatic duct at the anastomotic site, and in the intrahepatic bile duct, in Todani's type IV-A classification of congenital biliary dilation. 10 The development of intrahepatic cholangiocarcinoma after the "separation-operation" has not been reported in patients without dilation of the intrahepatic bile duct (such as those with Todani's type I congential biliary dilation).…”
mentioning
confidence: 99%
“…Only a few reports exist in the English literature [10][11][12][13]. No case has been reported in which all diagnostic procedures failed to delineate a communication between the cystic lesion containing carcinoma and the biliary tree.…”
Section: Discussionmentioning
confidence: 97%