Abstract
Nested stromal-epithelial tumor (NSET) is a rare and complex liver tumor characterized by multiple clustered nests of primitive-looking epithelial cells embedded in a spindle cell stroma, surrounded by a sheath of myofibroblastic cells, and associated with calcifications and ossification. The tumor occurs both in the pediatric age group and in adults and is not associated with preexisting liver disease. NSET may be associated with Cushing syndrome with production of ACTH due to ectopic synthesis of corticotropin-releasing hormone. NSET usually presents as a solitary liver neoplasm ranging in diameter from a few cm to 30 cm. A feature important for the elucidation of pathogenesis of NSET is the presence of nuclear beta-catenin expression in epithelial cells of the nests. This phenomenon is associated with mutations of the beta-catenin gene (exon 3) and expression of factors inducing epithelial-mesenchymal transition.
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Zimmermann, A. (2017). Nested Stromal-Epithelial Tumor of the Liver. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_23
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DOI: https://doi.org/10.1007/978-3-319-26956-6_23
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