Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: Report of a case and review of the literature

Maureen J. O'Sullivan, Paul E. Swanson, Joan Knoll, Eugenio M. Taboada, Louis P. Dehner

Research output: Contribution to journalArticle

86 Scopus citations

Abstract

Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule.

Original languageEnglish
Pages (from-to)482-489
Number of pages8
JournalPediatric and Developmental Pathology
Volume4
Issue number5
DOIs
StatePublished - Sep 12 2001

Keywords

  • Mesenchymal hamartoma
  • Mesothelium
  • Undifferentiated embryonal sarcoma

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