Abstract
One complication of choledochal cyst in adulthood is the development of secondary carcinoma, usually well-differentiated adenocarcinoma. We report a case of extrahepatic bile duct carcinoma associated with choledochal cyst and presenting predominantly sarcomatous features. The patient was a 52-year-old female who presented with abdominal fullness and pain. Ultrasound (US), computed tomography (CT), and cholangiography revealed a choledochal cyst with a tumor. The resected choledochal cyst was 10×7.5 cm and contained a protruding, ulcerated tumor, measuring about 4.0×2.5 cm. Histologically, the tumor was composed of spindle-shaped or fusiform cells with occasional pleomorphic or bizarre giant cells and abundant fibrous stroma, similar to malignant mesenchymal tumors. However, further histologic and immunohistochemical examination revealed that the tumor was adenocarcinoma with prominent sarcomatous features. To our knowledge, sarcomatous change in extrahepatic bile duct carcinoma associated with choledochal cyst has not been previously described.
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References
Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg 1980;140:653–657.
Todani T, Watanabe Y, Tokai A, et al. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 1987;164:61–64.
Mori W, Nagasako K. Cholangiocarcinoma and related lesions. In: Okuda K, Peters RL (eds) Hepatocellular carcinoma, New York: Wiley Medical, 1976;227–246.
Orloff MJ, Marassi NP. Tumors of the extrahepatic bile ducts. In: Berl JE, Haubrich WS, Kalser MH, Roth JLA, Schaffner F (eds) Bockus gastroenterology, vol 6. Philadelphia: WB Saunders, 1985;3771–3781.
Albores-Saavedra J, Henson DE. Tumors of the gallbladder and extrahepatic bile ducts. In: Hartmann WH and Sobin LH (eds). Atlas of tumor pathology. Second series, fascicle 22. Washington: Armed Forces Institute of Pathology, 1986; 164–187.
Albores-Saavedra J, Henson DE, Sobin LH. The WHO histological classification of tumors of the gallbladder and extrahepatic bile duct. A commentary on the second edition. Cancer 1992;70:410–414.
Nakajima T, Kondo Y, Miyazaki M, et al. A histopathologic study of 102 cases of intrahepatic cholangiocarcinoma: Histologic classification and modes of spreading. Hum Pathol 1988;19: 1228–1234.
Sasaki M, Nakanuma Y, Nagai Y, et al. Intrahepatic cholangiocarcinoma with sarcomatous transformation: An autopsy case. J Clin Gastroenterol 1991;13:220–225.
Yamaguchi K, Enjoji M. Carcinoma of the gallbladder. A clinicopathology of 103 patients and a newly proposed staging. Cancer 1988;62:1425–1432.
Albores-Saavedra J, Cruz-Ortiz H, Alcantara-Vazquez A, et al. Unusual types of gallbladder carcinoma. Arch Pathol Lab Med 1982;105:287–293.
Todani T, Watanabe Y, Marusue M, et al. Congenital bile duct cysts. Classification, operative procedures, and review of 37 cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263–269.
Nonomura A, Ohta G, Hayashi M, et al. Immunohistochemical detection of ras oncogene p21 product in liver cirrhosis and hepatocellular carcinoma. Am J Gastroenterol 1987;82:512–518.
Flanigan DP. Biliary carcinoma associated with biliary cyst. Cancer 1977;40:880–883.
Todani T, Tabuchi K, Watanabe Y, et al. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979;44:1134–1141.
Osborn M, Weber K. Biology of disease. Tumor diagnosis by intermediate filaments typing: A novel tool for surgical pathology. Lab Invest 1986;48:372–394.
Azumi N, Battifora H. The distribution of vimentin and keratin in epithelial and nonepithelial neoplasms. Am J Clin Pathol 1987;88:286–296.
Kakizoe S, Kojiro M, Nakashima T. Hepatocellular carcinoma with sarcomatous change: Clinicopathologic and immunohistochemical studies of 14 autopsy cases. Cancer 1987;59: 310–316.
Nakajima T, Kubosawa H, Kondo Y, et al. Combined hepatocellular-cholangiocarcinoma with variable sarcomatous transfromation. Am J Clin Pathol 1988;90:309–312.
Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977;67:40–46.
Gupta S, Kumar A, Gupta SK, et al. Carcinoma in choledochal cyst. J Surg Oncol 1981;16:313–318.
Ritchie JK, Allan RN, Macartney J, et al. Biliary tract carcinoma associated with ulcerative colitis. Q J Med 1974;43:263–267.
Hodgson HJF, Davies DR, Thompson RPH. Congenital hepatic fibrosis. J Clin Pathol 1976;29:11–16.
Dayton MT, Longmire WP Jr, Tompkins RK. Caroli's disease: A premalignant condition? Am J Surg 1983;145:41–48.
Polk HC. Carcinoma and the calcified gallbladder. Gastroenterology 1966;50:582–585.
Kulkarni PB, Beatty EC. Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. Am J Dis Child 1977;131:442–446.
Donaldson DR, Guillon PJ. Extrahepatic cholangiocarcinoma in association with long-standing cholangitis. Br J Clin Pract 1979;33:297–300.
Fortner JG. An appraisal of the pathogenesis of primary carinoma of the extrahepatic bile duct. Surgery 1958;43:563–568.
Spitz L, Petropoulos A. The development of the glands of the common bile duct. J Pathol 1979;138:213–220.
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Nonomura, A., Mizukami, Y., Matsubara, F. et al. A case of choledochal cyst associated with adenocarcinoma exhibiting sarcomatous features. J Gastroenterol 29, 669–675 (1994). https://doi.org/10.1007/BF02365455
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DOI: https://doi.org/10.1007/BF02365455