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WHO Classification of Tumours
Calcifying nested epithelial stromal tumor
Liver and intrahepatic bile ducts


This neoplasm is an unusual, probably favourable hepatic tumour in children of young to older age
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Heerema-McKenney A, Leuschner I, Smith N, Sennesh J, Finegold MJ (2005)
Nested stromal epithelial tumor of the liver: six cases of a distinctive pediatric neoplasm with frequent calcifications and association with cushing syndrome.
Am J Surg Pathol 29: 10-20

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Hill DA, Swanson PE, Anderson K, Covinsky MH, Finn LS, Ruchelli ED, Nascimento AG, Langer JC, Minkes RK, McAlister W, Dehner LP (2005)
Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity.
Am J Surg Pathol 29: 1-9

. It is characterized histologically by nested spindle and epithelioid cells and sometimes marked desmoplasia. The nests of epithelioid cells resemble immature, keratin 8- and epithelial membrane antigenpositive hepatoid cells with eosinophilic cytoplasm. These nests are surrounded by spindle cells that stain for vimentin and α-smooth muscle actin. Calcifications and bone formation may occur. A possible relationship to mixed blastomatous tumours of the paediatric liver is uncertain, but, like hepatoblastomas, the epithelioid cells in nested tumours have nuclear positivity for β-catenin, suggesting a pathogenic role for mutations in the β-catenin gene
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010