Beta-catenin mutation does not seem to have an effect on the tumorigenesis of pediatric rhabdomyosarcomas.
Involvement of the Wnt signal transduction pathway has been shown in different pediatric embryonal tumors, such as hepatoblastoma, nephroblastoma, pancreatoblastoma, and medulloblastoma. There are few data available on the status of beta-catenin in rhabdomyosarcoma (RMS), another pediatric embryonal tumor. The aims of this study were 1st to verify the status of the exon 3 of CTNNB1 and 2nd to assess the usefulness of beta-catenin immunostaining in a small series of 8 embryonal RMS, 3 alveolar RMS, and 1 sclerosing RMS (SRMS). Sequence analysis revealed no mutations in the exon 3 of CTNNB1 in all the tumors studied. All RMS showed a cytoplasmic beta-catenin staining with cytoplasmic membrane reinforcement and no nuclear delocalization. We conclude that there is no evidence of beta-catenin mutation in the genesis of rhabdomyosarcoma and that beta-catenin does not represent a useful immunomarker to help distinguish between embryonal RMS and alveolar RMS.
Bouron-Dal Soglio
Dorothée
D
Departments of Pathology, CHU Sainte-Justine, 3175 chemin de la Côte Sainte-Catherine, Montréal, QC, Canada. dorothee.dal_soglio.hsj@ssss.gouv.qc.ca
Rougemont
Anne-Laure
AL
Absi
Riwa
R
Giroux
Louise-Marie
LM
Sanchez
Rocio
R
Barrette
Stéphane
S
Fournet
Jean-Christophe
JC
D000293:Adolescent; D014408:Biomarkers, Tumor / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D009154:Mutation; D016133:Polymerase Chain Reaction; D018232:Rhabdomyosarcoma, Alveolar / Q000235:genetics* / Q000473:pathology*; D018233:Rhabdomyosarcoma, Embryonal / Q000235:genetics* / Q000473:pathology*; D051176:beta Catenin / Q000235:genetics*
D014408:Biomarkers, Tumor; D051176:beta Catenin