Congenital choledochal cyst is occasionally complicated by carcinomatous transformation, mostly adenocarcinoma. Adenosquamous carcinoma arising in a congenital choledochal cyst is very rare. The author herein reports an adenosquamous carcinoma arising in congenital choledochal cyst associated with pancreatico-biliary maljunction. A 34-year-old man with congenital choledochal cyst and recurrent cholangitis had been followed up, and was admitted to hospital to undergo testing for cancer. Imaging modalities including computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiography showed an elevated lesion in the choledochal cyst. Because clinical cytology of bile indicated malignant cells, pancreatico-duodenectomy, cholecystectomy, and resection of the choledochal cyst were performed. Grossly, the choledochal cyst was type I, and its size was 8 x 10 cm. Anomalous pancreatico-biliary ductal union was recognized. An elevated lesion was recognized in the choledochal cyst. Histologically, the lesion was composed of a squamous cell carcinoma element and an adenocarcinoma element; a gradual transition was recognized between the two. The squamous cell carcinoma element contained microcytic cells with mucins. On immunohistochemistry the adenocarcinoma element and microcytic cells were positive for CEA, but the squamous cell carcinoma element was negative for CEA. Both elements were positive for CA19-9. Ki-67 labeling was 53% in the adenocarcinoma element and 48% in the squamous cell carcinoma element. p53 protein was negative in both elements. At the time of writing, the patient was alive after 25 months without recurrence or metastasis. The present case is the second case of adenosquamous carcinoma arising in congenital choledochal cyst in the English-language literature.
[Show abstract][Hide abstract] ABSTRACT: Adenosquamous carcinoma arising in congenital choledochal cyst is very rare and herein we report a case thereof. A 37-year-old woman was referred for further evaluation of pancreas head mass and a hepatic nodule on CT. She had been diagnosed with congenital choledochal cyst at 22-years-old and received Roux-en-Y choledochojejunostomy at that time. Endoscopic ultrasonography-guided biopsy proved the pancreas head mass as a squamous cell carcinoma and liver biopsy also proved the liver mass as a metastatic squamous cell carcinoma. We performed pancreaticoduodenectomy and tumorectomy of metastatic liver nodule. Grossly, the primary lesion was located at intrapancreatic portion of choledochal cyst. Histologically, the primary lesion and hepatic nodule was metastatic adenosquamous carcinoma. So far, there have been only three cases of adenosquamous carcinoma arising in congenital choledochal cyst reported in English-language literature. This is another case and the first case reported in Korea.
Journal of the Korean Surgical Society 05/2010; 78(5). DOI:10.4174/jkss.2010.78.5.325 · 0.73 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background:
To retrospectively review the MRI imaging features of adult choledochal cysts associated with biliary malignancy.
Patients and methods:
Ten out of 72 cases of adult choledochal cysts were found to be associated with biliary malignancy between January 1, 2003 and April 1, 2011 in our hospital database. The following MRI findings of these ten patients were retrospectively reviewed: the type of choledochal cysts, the presence of anomalous union of the pancreaticobiliary duct (AUPBD), manifestations of biliary malignancy, and concomitant findings.
Among the ten patients, there were five type I and five type IVA choledochal cysts. AUPBD was noted in four cases. The biliary malignancy was diagnosed as cholangiocarcinoma in seven cases (70.0%) and as gallbladder cancer in three cases. Cholangiocarcinoma manifested with irregularly thickened cyst wall (n = 2), mass with irregularly thickened cyst wall (n = 4), or multiple papillary nodules without thickened cyst wall (n = 1). Most of them showed mark enhancement (n = 4) after contrast administration. Gallbladder cancer appeared as mass with irregular thickening of the gallbladder wall with inhomogeneous enhancement. Concomitant findings included liver invasion or metastases in five cases, lymph node metastases in two cases, cholangitis and/or hepatic abscess in two cases, biliary stones in three cases. The type of choledochal cysts and the extent of malignant tumor invasion revealed by MRI were consistent with the surgical findings.
Most malignancies associated with choledochal cysts are cholangiocarcinoma and gallbladder cancer. MRI is a reliable method for the detection of choledochal cysts with biliary malignant changes. MR features such as irregular thickening of the gallbladder wall or cyst wall, mass or papillary nodules are suggestive of biliary malignant changes.
[Show abstract][Hide abstract] ABSTRACT: Abstract We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.
International surgery 10/2012; 97(4):315-20. DOI:10.9738/CC152.1 · 0.47 Impact Factor
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