Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma

Ramapriya Ganti, Stephen X. Skapek, Jie Zhang, Christine E. Fuller, Jianrong Wu, Catherine A. Billups, Philip P. Breitfeld, James D. Dalton, William H. Meyer, Joseph D. Khoury

Research output: Contribution to journalArticlepeer-review

68 Scopus citations


Both epidermal growth factor receptor (EGFR) and ErbB-2 play an important role in cancer biology and constitute promising molecular targets of therapy. EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors. Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2. Immunostains were assessed for intensity (0: no immunostaining; 1: weak; 2: moderate; 3: strong) and percentage of at least 500 neoplastic cells exhibiting membranous or membranous and cytoplasmic immunostaining. EGFR and ErbB-2 expression was considered positive if the product of intensity and percentage was greater than 10. Patients had a median age of 5.7 years (range 8 months-19.1 years), and of 65/66 patients, 38 were males and 27 were females. Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P=0.30). Expression of EGFR was identified in 31/66 (47%) cases and correlated with the embryonal subtype (26/34, 76%, vs 5/32, 16%, P<0.0001) independent of stage, age, and gender. Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma. None of the cases exhibited EGFR or ErbB-2 gene amplification, as assessed using fluorescence in situ hybridization. Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21. In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain. Notably, expression of EGFR correlates with the embryonal subtype, which is also more likely to coexpress EGFR and ErbB-2.

Original languageEnglish
Pages (from-to)1213-1220
Number of pages8
JournalModern Pathology
Issue number9
StatePublished - Sep 2 2006

Bibliographical note

Funding Information:
We acknowledge the outstanding services of St Jude Children’s Research Hospital’s Tissue Resources Facility and the Hartwell Center for Bioinformatics and Biotechnology. This work was supported in part by NIH grant CA-023099 (JDK) and by the American Lebanese Syrian Associated Charities (ALSAC).


  • Epidermal growth factor receptor
  • ErbB-2
  • Fluorescence in situ hybridization
  • Immunohistochemistry
  • Rhabdomyosarcoma
  • Tissue microarray

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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