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Bile Duct Carcinomas with Marked Extracellular or Intracellular Mucin Accumulation: Mucinous and Signet Ring Cell Carcinomas

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract
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Abstract

Uncommon variants of cholangiocarcinoma, both of intrahepatic and extrahepatic ducts, are characterized by marked production of mucin. Depending on secretion or storage of mucins, two major groups of mucin-rich carcinomas are distinguished, i.e., mucinous cholangiocarcinoma or signet ring cell carcinoma. Mucinous cholangiocarcinoma (colloid carcinoma) is a rare cancer that may develop as a complication of hepatolithiasis. A part of these neoplasms also occur in the setting of intraductal papillary neoplasms of bile ducts. Most mucinous cholangiocarcinomas are intrahepatic neoplasms, but very rare forms also occur in extrahepatic ducts and cystic duct. Due to massive extracellular mucin accumulation, mucinous cholangiocarcinomas can show rapid mass increase. In contrast to mucinous cancers, the rare signet ring cell carcinomas of bile duct do not secrete mucin, but store it within cells, resulting in intracellular mucin deposits with peripheral displacement of the nucleus. Signet ring cell carcinomas show a diffuse growth pattern and are highly invasive lesions.

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Zimmermann, A. (2017). Bile Duct Carcinomas with Marked Extracellular or Intracellular Mucin Accumulation: Mucinous and Signet Ring Cell Carcinomas. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_35

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