title: Undifferentiated (embryonal) sarcoma arising in conjunction with mesenchymal hamartoma of the liver.
abstract: The histogenesis of the undifferentiated (embryonal) sarcoma of the liver has been much debated. Originally, some investigators suggested that the tumor could originate from a mesenchymal hamartoma. Others doubted this hypothesis. The demonstration of alpha-1-antitrypsin in some tumors and of cytokeratins in others gave rise to new theories suggesting that the tumor was either a fibrous histiocytoma or a sarcomatoid hepatocellular carcinoma. As a new development in this search for the genesis of the undifferentiated (embryonal) sarcoma of the liver, this report describes a case that seems to substantiate, for the first time, the original hypothesis histogenetically linking the undifferentiated (embryonal) sarcoma and the mesenchymal hamartoma. The case is that of a 12-yr-old girl who developed an undifferentiated (embryonal) sarcoma of the liver in conjunction with a mesenchymal hamartoma. Furthermore, in places, the tumor exhibited striking epithelial differentiation as well as immunoreactivity for cytokeratins, alpha-1-antitrypsin and vimentin. Immunoreactivity for alpha-fetoprotein was limited to the areas of epithelial differentiation.
authors.affiliation: Department of Pathology, Temple University School of Medicine, Philadelphia, Pennsylvania.
authors.forename: J P
authors.initials: JP
authors.lastname: de Chadarévian
authors.forename: B R
authors.initials: BR
authors.lastname: Pawel
authors.forename: E N
authors.initials: EN
authors.lastname: Faerber
authors.forename: W H
authors.initials: WH
authors.lastname: Weintraub
mesh_terms: D002648:Child; D005260:Female; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000002:abnormalities*; D008113:Liver Neoplasms / Q000473:pathology*; D008648:Mesoderm / Q000473:pathology; D008853:Microscopy; D009373:Neoplasms, Germ Cell and Embryonal / Q000473:pathology*; D011230:Precancerous Conditions / Q000473:pathology*