Originally published as JCO Early Release 10.1200/JCO.2005.03.4934 on January 3 2006

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Subtype and Prognostic Classification of Rhabdomyosarcoma by Immunohistochemistry

From the University Children's Hospital, Division of Oncology; University Hospital Zurich, Department of Pathology, Zurich, Switzerland; University of Kiel, Department of Pathology, Kiel; and Olga Hospital, Department of Pediatric Oncology/Hematology, Stuttgart, Germany

Address reprint requests to Beat W. Schäfer, PhD, Department of Oncology, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland; e-mail: beat.schaefer{at}kispi.unizh.ch

PURPOSE: Rhabdomyosarcoma (RMS) is classified into two main subgroups: the embryonal (ERMS) and the alveolar (ARMS) form. The majority of the ARMSs are associated with specific chromosomal translocations (pARMS). Because ARMS is much more aggressive than ERMS, RMS subclassification has clinical relevance. However, diagnosis of RMS subgroups on the basis of histology or molecular biology can be difficult, and supplementing diagnostic methods would be desirable. The aim of this study was to establish a panel of markers for RMS subgroup classification by immunohistochemistry.

MATERIALS AND METHODS: Gene expression data were used for selection of subgroup-specific markers. Single sections of RMS with available expression data were used for establishment of the immunohistochemistry. Evaluation of the sensitivity and specificity of the markers was carried out using a tissue array representing 252 RMSs. Kaplan-Meier survival curves were calculated for determination of differences in overall survival of the different staining subgroups.

RESULTS: AP2ß and P-cadherin were selected as markers for pARMS, and epidermal growth factor receptor (EGFR) and fibrillin-2 as markers for ERMS. EGFR + fibrillin-2 detected ERMS with a specificity of 90% and with a sensitivity of 60%. AP2ß + P-cadherin detected pARMS with a specificity of 98% and a sensitivity of 64%, and allowed the detection of several misclassified tumors. The EGFR + fibrillin-2–positive group is associated with a favorable outcome, and the AP2ß + P-cadherin–positive group is associated with an unfavorable outcome.

CONCLUSION: The presented set of marker proteins detects RMS subgroups with high specificity and may be useful in routine subtype classification of RMS.

Supported by a grant from the University Zurich, and by Oncosuisse (Grant No. 01473-02-2004), Krebsliga of the Kanton Zug, and by the Schweizer Forschungsstiftung Kind + Krebs.

Terms in blue are defined in the glossary, found at the end of this article and online at www.jco.org.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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