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Malignant Rhabdoid Tumors and Tumors with Rhabdoid Features

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

Abstract

Several tumors are characterized by the presence of a distinct cell type, the rhabdoid cell. This cell of still unknown origin shows a striking cytoskeletal inclusion that is visualized as an eosinophilic eccentrically placed cell body. Certain neoplasms, malignant rhabdoid tumors, are entirely composed of rhabdoid cells, while numerous other tumors contain subsets of rhabdoid cells or rhabdoid-like cells (neoplasms with rhabdoid features). The presence of rhabdoid cells generally confers a highly malignant phenotype. Rhabdoid tumors consistently show absence of nuclear reactivity for the chromatin-remodeling factor, SRF5/INI1. Malignant rhabdoid tumor is a well-known cancer of the kidney, but it also rarely occurs as a primary tumor in the liver of infants and children and exceptionally in adolescents and adults. The liver can also be involved in a rare and fatal condition of early infancy, congenital disseminated malignant rhabdoid tumor. There are few other complex pediatric liver neoplasms with rhabdoid cell elements. In adults, several hepatobiliary neoplasms, including cholangiocarcinoma, can exhibit rhabdoid features.

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    Arthur Zimmermann

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Zimmermann, A. (2017). Malignant Rhabdoid Tumors and Tumors with Rhabdoid Features. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_78

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