β-Catenin mutations in 2 nested stromal epithelial tumors of the liver—a neoplasia with defective mesenchymal-epithelial transition

摘要: Nested stromal epithelial tumor of the liver is a rare neoplasm early childhood and adolescence with characteristic nested morphology spindle epithelioid cells. Histogenesis pathogenesis this are, however, still unclear. Because mesenchymal cells suggestive altered mesenchymal-epithelial transition β-catenin mutations are rather common in other tumors such as hepatoblastomas, we investigated gene 2 analyzed additional factors involved transition, E-cadherin, vimentin, c-Met, TWIST, SNAIL, SLUG by molecular genetic immunohistochemical methods. Mutation analysis both cases revealed large deletions exon 3 (155 228 base pairs), resulting an accumulation cytoplasm nuclei cells, evidenced immunohistochemistry. The expression SLUG, was generally increased, whereas E-cadherin decreased. Morphological analysis, showed variable pattern various markers cell compartments tumors, thus illustrating transitional status In conclusion, our data clearly identify protein stabilizing feature liver, similarly hepatoblastomas. Therefore, may be regarded variant hepatoblastoma, despite differing from it clinical morphological aspects. epithelioid-spindle along incomplete differentiation proposes impaired possible pathogenetic mechanism tumor. However, because only were studied, hypothesis awaits further validation.