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


Definition

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



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