Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: A children's oncology group study

Kathleen Nicol, Van Savell, Julie Moore, Lisa Teot, Sheri L. Spunt, Stephen Qualman, Richard J. Andrassy, Carola A S Arndt, K. Scott Baker, Frederic G. Barr, W. Archie Bleyer, Philip Breitfeld, John C. Breneman, Julia Bridge, Kenneth Brown, Sarah S. Donaldson, Holcome E. Grier, Douglas Hawkins, Peter J. Houghton, Michael LinkThom L. Lobe, Harold M. Maurer, William H. Meyer, Jeff Michalski, Charles N. Paidas, Alberto S. Pappo, David M. Parham, R. Beverly Raney, Leslie Robison, Eric Sandler, Stephen Skapek, Lynn Smith, Paul H B Sorenson, David Walterhouse, Moody Wharam, Eugene S. Wiener, Suzanne Wolden, Richard Womer

Research output: Contribution to journalArticle

39 Scopus citations

Abstract

Morphologically, the distinction between undifferentiated embryonal sarcoma of the liver (UESL) and biliary tract rhabdomyosarcoma (RMS) can be uncertain because of some shared pathologic similarities. Patients with UESL have been consistently but erroneously enrolled in Children's Oncology Group (COG) treatment protocols because UESL was equated with RMS, despite the differing primary treatment modalities of these entities. Review of COG pathology files yielded 20 cases of UESL that were compared to 25 cases of biliary tract RMS. Clinicopathologic features including immunohistochemical staining were examined. In the UESL cases, the male:female ratio was 1:1 and the median age was 10.5 years. Histologically, hyaline globules and diffuse anaplasia were consistently present. The cases of RMS had a male:female ratio of 1.8:1 with a median age of 3.4 years and routinely lacked diffuse anaplasia and hyaline globules. Polyclonal desmin and muscle-specific actin were variably immunoreactive in UESL and RMS; however, myogenin and myogenic regulatory protein D1 (MyoD1) were uniformly negative in UESL and routinely positive in the majority of biliary tract RMS. Myogenin, in particular, was highly significant (P = 0.0003) in distinguishing RMS from UESL. With a median follow-up of 8 months, 11 of 18 patients with UESL were still alive. The estimated 5-year survival for biliary tract RMS was 66%. Establishing the correct diagnosis of these distinct clinical and pathologic entities is important, as surgery alone may be curative in UESL, whereas initial chemotherapy is often recommended for the treatment of biliary tract RMS.

Original languageEnglish (US)
Pages (from-to)89-97
Number of pages9
JournalPediatric and Developmental Pathology
Volume10
Issue number2
DOIs
StatePublished - Mar 2007

Keywords

  • Biliary tract
  • Child
  • Liver
  • Pediatric
  • Rhabdomyosarcoma
  • Undifferentiated embryonal sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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