Adenosquamous carcinoma in a congenital choledochal cyst associated with pancreatico-biliary maljunction

Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
Pathology International (Impact Factor: 1.69). 08/2009; 59(7):482-5. DOI: 10.1111/j.1440-1827.2009.02397.x
Source: PubMed


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.

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    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.
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    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.
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