Article

Myogenin is a Specific Marker for Rhabdomyosarcoma: An Immunohistochemical Study in Paraffin-Embedded Tissues.

Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda Maryland, USA.
Modern Pathology (Impact Factor: 6.36). 10/2000; 13(9):988-93. DOI: 10.1038/modpathol.3880179
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ABSTRACT Myogenin belongs to a group of myogenic regulatory proteins whose expression determines commitment and differentiation of primitive mesenchymal cells into skeletal muscle. The expression of myogenin has been demonstrated to be extremely specific for rhabdomyoblastic differentiation, which makes it a useful marker in the differential diagnosis of rhabdomyosarcomas (RMS) from other malignant small round cell tumors of childhood. Commercially available antibodies capable of detecting myogenin in routinely processed formalin-fixed paraffin-embedded (FFPE) tissue are now available. In this study, we evaluated myogenin expression using the monoclonal myf-4 antibody (Novocastra Labs) on FFPE in a large number of pediatric tumors in order to define the clinical utility of this marker. A total of 119 tumors were studied. These included 48 alveolar RMS (ARMS), 20 embryonal RMS (ERMS), one spindle cell RMS, 16 Ewing's sarcomas (ES), six nephroblastomas, two ectomesenchymomas, seven precursor hematopoietic neoplasms, five olfactory neuroblastomas, three neuroblastomas, six desmoplastic small round cell tumors, and five rhabdoid tumors. Distinct nuclear staining for myogenin was noted in all 69 RMS. Notably, the number of positive tumor cells differed between the ARMS and ERMS. In ARMS, the majority of tumor cells (75 to 100%) were positive, in contrast to ERMS, in which the positivity ranged from rare + to 25% in all but three tumors. Additionally, myogenin positivity was seen in two of two ectomesenchymomas and in two nephroblastomas with myogenous differentiation. All other tumors were clearly negative. Our results indicate that staining for myogenin is an extremely reliable and specific marker for rhabdomyoblastic differentiation. It gives consistent and easily interpretable results in routinely fixed tissues.

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    • "As neither of these markers is sufficiently specific (Cerilli and Wick, 2002 ; Coindre, 2003; Hasegawa et al .. 2003 ; Kodet, 1989, Ordonez, 1998; Truong et al ., 1990), they were complemented by myogenin, a 263 muscular marker considered as extremely sensitive and reported as specific for identifying human rhabdomyosarcomas (Cessna et al ., 2001 ; Coindre, 2003 ; Cui et al ., 1999 ; Kumar et al ., 2000). It was shown (Coindre, 2003) that more than 90% of human rhabdomyosarcomas are positive for myogenin and that the non-rhabdomyosarcomas are all clearly negative (Kumar et al ., 2000) or only show rare focal reactivity (Cessna et al., 2001). Myogenin belongs to a group of myogenic regulatory proteins whose expression determines commitment and differentiation of primitive mesenchyrnal cells into skeletal muscle and is also expressed in regenerative skeletal muscle cells . "
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