Several types of biliary cystic tumors or tumorous lesions have been reported (Nakanuma et al., 2000; Portman and Nakanuma, 2006). Currently, with advanced imaging techniques, increasing numbers of cystic diseases including rare types are being found, and their clinicopathological features are now being characterized (Okuda, 2001). Hepatobiliary cystadenoma and cystadenocarcinoma are a prototype of biliary cystic tumors, and intraductal papillary neoplasm of the bile duct, particularly with cystic dilatation, is a recently proposed disease entity (Chen et al., 2001). However, the exact definition and clinicopathological features of these biliary cystic tumors, particularly for differential diagnosis, remain to be established because the number of cases is small. While the cystic lesions themselves are rather easily detectable by imaging modalities, an exact diagnosis based on pathological characteristics is needed at present.
In this chapter, the clinicopathological features of biliary cystic tumors, such as their walls being lined by biliary or other epithelial cells, are reviewed. The tumors can be divided into non-neoplastic and neoplastic categories. The former includes hamartomatous lesions or developmental anomalies. Recently, diagnostic criteria for intraductal papillary mucinous neoplasm (IPMN) and mucinous cystadenoma and cystadeno-carcinoma, which are also called mucinous cystic neoplasm of the pancreas (pancreatic MCN), have been established with reference to the presence of communication with the duct system and also the presence of ovarian-like stroma in the cystic walls (Longnecker et al., 2000; Zamboni et al., 2000). Recent studies showed that two similar types of neoplastic diseases are also present in the hepatobiliary system (Chen et al., 2001; Zen et al., 2006b; Abraham et al., 2002, 2003). In this review, these biliary cystic tumors are discussed and compared with pancreatic cystic tumors from the standpoint of their clinicopathological similarities. Solitary hepatic cyst, polycystic liver, and cystic dilatation of the bile duct will not be discussed here.
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References
Abraham, S.C., Lee, J.H., Hruban, R.H., Argani, P., Furth, E.E., and Wu, T.T. 2003. Molecular and immunohistochemical analysis of intraductal papillary neoplasms of the biliary tract. Hum. Pathol. 34: 902–910.
Abraham, S.C., Lee, J.H., Boitnott, J.K., Argani, P., Furth, E.E., and Wu, T.T. 2002. Microsatellite instability in intraductal papillary neoplasms of the biliary tract. Mod. Pathol. 15: 1309–1317.
Adbores-Saavedra, J., Scoazec, J.C., Wittekind, C., Sripa, B., Menck, H.R., Soehendra, N., and Sriram, P.V.J. 2000. Carcinoma of the gallbladder and extrahepatic bile ducts. In: Hamilton, S.R., Aaltonen, L.A., eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC Press, 207–214.
Ashida, K., Terada, T., Kitamura,Y., and Kaibara, N. 1998. Expression of E-cadherin, alpha-catenin, beta-catenin, and CD44 (standard and variant iso-forms) in human cholangiocarcinoma: an immunohistochemical study. Hepatology 27:974–82.
Chen, T.C., Nakanuma, Y., Zen, Y., Chen, M.F., Jan, Y.Y., Yeh, T.S., Chiu, C.T., Kuo, T.T., Kamiya, J., Oda, K., Hamaguchi, M., Ohno, Y., Hsieh, L.L., and Nimura, Y. 2001. Intraductal papillary neoplasia of the liver associated with hepatolithiasis. Hepatology 34: 651–658.
DeHaan, R.D., Kipp, B.R., Smyrk, T.C., Abraham, S.C., Roberts, L.R., and Halling, K.C. 2007. An assessment of chromosomal alterations detected by fluorescence in situ hybridization and p16 expression in sporadic and primary sclerosing cholangitis-associated cholangiocarcinomas. Hum. Pathol. 38: 491–499.
Devaney, K., Goodman, Z.D., and Ishak, K.G. 1994. Hepatobiliary cystadenoma and cystade-nocarcinoma. A light microscopic and immuno-histochemical study of 70 patients. Am. J. Surg. Pathol. 18: 1078–1091.
Fujii, T., Harada, K., Katayanagi, K., Kurumaya, H., and Nakanuma, Y. 2005. Intrahepatic cholangi-ocarcinoma with multicystic, mucinous appearance and oncocytic change. Pathol. Int. 55: 206–209.
Hamazaki, K., Gochi, A., Shimamura, H., Kaihara, A., Maruo, Y., Doi, Y., Orita, K., Lygidakis, N.J. 1996. Serum levels of circulating intercellular adhesion molecule 1 in hepatocellular carcinoma. Hepatogastroenterology 43: 229–234.
Ishikawa, A., Sasaki, M., Ohira, S., Ohta, T., Oda, K., Nimura, Y., Chen, M.F., Jan, Y.Y., Yeh, T.S., and Nakanuma, Y. 2004a. Aberrant expression of CDX2 is closely related to the intestinal metaplasia and MUC2 expression in intraductal papillary neoplasm of the liver in hepatolithiasis. Lab. Invest. 84: 629–638.
Ishikawa, A., Sasaki, M., Sato, Y., Ohira, S., Chen, M.F., Huang, S.F., Oda, K., Nimura, Y., and Nakanuma, Y. 2004b. Frequent p16ink4a inac-tivation is an early and frequent event of intraductal papillary neoplasm of the liver arising in hepatolithiasis. Hum. Pathol. 35: 1505–1514.
Kadoya, M., Matsui, O., Nakanuma, Y., Yoshikawa, J., Arai, K., Takashima, T., Amano, M., and Kimura, M. 1990. Ciliated hepatic foregut cyst: radiologic features. Radiology 175: 475–477.
Kida, T., Nakanuma, Y., and Terada, T. 1992. Cystic dilatation of peribiliary glands in livers with adult polycystic disease and livers with solitary nonparasitic cysts: an autopsy study. Hepatology 16: 334–340.
Lee, S.S., Kim, M.H., Lee, S.K., Jang, S.J., Song, M.H., Kim, K.P., Kim, H.J., Seo, D.W., Song, D.E., Yu, E., Lee, S.G., and Miin, Y.I. 2004. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer 100: 783–793.
Longnecker, D.S., Hruban, R.H., Adler, G., and Kloppel, G. 2000. Intraductal papillary-mucinous neoplasms of the pancreas. In: Hamilton, S.R., Aaltonen, L.A., eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC Press, 237–240.
Nakanuma, Y., Kurumaya, H., and Ohta, G. 1984. Multiple cysts in the hepatic hilum and their pathogenesis. A suggestion of periductal gland origin. Virchows. Arch. A. Pathol. Anat. Histopathol. 404: 341–350.
Nakanuma, Y., Hoso, M., Sanzen, T., and Sasaki, M. 1997. Microstructure and development of the normal and pathologic biliary tract in humans, including blood supply. Microsc. Res. Tech. 38: 552–570.
Nakanuma, Y., Leong, A.S.Y., Sripa, B., Ponchon, T., Vatanasapt, V., and Ishak, K.G. 2000. Intrahepatic cholangiocarcinoma. In: Hamilton, S.R., Aaltonen, L.A., eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC Press, 173–180.
Nakanuma, Y. 2004. Peribiliary cysts have at least two different pathogeneses. J. Gastroenterol. 39: 407–408.
Okuda, K. 2001. Okuda, K., Mitchell, D.G., Itai, Y., Ariyama, J., eds. Hepatobiliary Diseases. Oxford: Blackwell Science.
Portman, B.C., and Nakanuma, Y. 2006. Diseases of bile ducts. Burt, A.D., Portman, B.C. Ferrell, L.D., eds. MacSween's Pathology of the Liver. Churchill Livingstone 5th ed., pp. 517–581.
Qian, Q., Li, A., King, B.F., Kamath, P.S., Lager, D.J., Huston, J., 3rd, Shub, C., Davila, S., Somlo, S., and Torres, V.E. 2003. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 37: 164–71.
Sato, Y., Kitagawa, S., Zen, Y., Minato, H., and Nakanuma, Y. 2006. Ciliated hepatic cyst without smooth muscle layer: a variant of ciliated hepatic foregut cyst? Pathol. Int. 56: 340–344.
Sato, M., Watanabe, Y., Tokui, K., Kohtani, T., Nakata, Y., Chen, Y., and Kawachi, K. 2003. Hepatobiliary cystadenocarcinoma connected to the hepatic duct: a case report and review of the literature. Hepatogastroenterology 50: 1621–1624.
Sudo, Y., Harada, K., Tsuneyama, K., Katayanagi, K., Zen, Y., and Nakanuma, Y. 2001. Oncocytic biliary cystadenocarcinoma is a form of intra-ductal oncocytic papillary neoplasm of the liver. Mod. Pathol. 14: 1304–1309.
Terada, T., and Nakanuma, Y. 1990a. Pathological observations of intrahepatic peribiliary glands in 1,000 consecutive autopsy livers. III. Survey of necroinflammation and cystic dilatation. Hepatology 12: 1229–1233.
Terada, T., Nakanuma, Y., and Ohta, G. 1987. Glandular elements around the intrahepatic bile ducts in man; their morphology and distribution in normal livers. Liver 7: 1–8.
Terada, T., Nakanuma, Y., Kono, N., Ueda, K., Kadoya, M., and Matsui, O. 1990. Ciliated hepatic foregut cyst. A mucus histochemical, immunohistochemical, and ultrastructural study in three cases in comparison with normal bronchi and intrahepatic bile ducts. Am. J. Surg. Pathol. 14: 356–363.
Terada, T., and Nakanuma, Y. 1990b. Pathological observations of intrahepatic peribiliary glands in 1,000 consecutive autopsy livers. II. A possible source of cholangiocarcinoma. Hepatology 12: 92–97.
Wheeler, D.A., and Edmondson, H.A. 1985. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change. Cancer 56: 1434–1445.
Zamboni, G., Longnecker, D.S., Kloppel, G., Adler, G., and Hruban, R.H. 2000. Mucinous cystic neoplasm of the pancreas. In: Hamilton SR, Aaltonen LA, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC Press, pp. 234–236.
Zen, Y., Fujii, T., Itatsu, K., Nakamura, K., Konishi, F., Masuda, S., Mitsui, T., Asada, Y., Miura, S., Miyayama, S., Uehara, T., Katsuyama, T., Ohta, T., Minato, H., and Nakanuma, Y. 2006a. Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct. Mod. Pathol. 19: 1243–1254.
Zen, Y., Fujii, T., Itatsu, K., Nakamura, K., Minato, H., Kasashima, S., Kurumaya, H., Katayanagi, K., Kawashima, A., Masuda, S., Niwa, H., Mitsui, T., Asada, Y., Miura, S., Ohta, T., and Nakanuma, Y. 2006b. Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas. Hepatology 44: 1333–1343.
Zen, Y., Terahata, S., Miyayama, S., Mitsui, T., Takehara, A., Miura, S., Nobata, K., Kitao, A., Kakuda, K., Kiyohara, K., and Nakanuma, Y. 2006c. Multicystic biliary hamartoma: a hitherto undescribed lesion. Hum. Pathol. 37: 339–344.
Zen, Y., Sasaki, M., Fujii, T., Chen, T.C., Chen, M.F., Yeh, T.S., Jan, Y.Y., Huang, S.F., Nimura, Y., and Nakanuma, Y. 2006d. Different expression patterns of mucin core proteins and cytokeratins during intrahepatic cholangiocar-cinogenesis from biliary intraepithelial neoplasia and intraductal papillary neoplasm of the bile duct — an immunohistochemical study of 110 cases of hepatolithiasis. J. Hepatol. 44: 350–358.
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Nakanuma, Y., Ikeda, H., Sato, Y., Harada, K., Nakamura, K., Zen, Y. (2009). Biliary Cystic Tumors: Clinicopathological Features. In: Hayat, M.A. (eds) Liver Cancer. Methods of Cancer Diagnosis, Therapy and Prognosis, vol 5. Springer, Dordrecht. https://doi.org/10.1007/978-1-4020-9804-8_31
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