title: Oncofetal expression of Wilms' tumor 1 (WT1) protein in human fetal, adult and neoplastic skeletal muscle tissues.
abstract: There is increasing evidence that WT1 protein expression is found not only at nuclear, but also at cytoplasmic, level in several developing and neoplastic tissues. In order to better understand the possible role of WT1 protein in human skeletal myogenesis and oncogenesis of rhabdomyosarcoma, we assessed immunohistochemically its comparative expression in a large series of human developing, adult and neoplastic skeletal muscle tissues. The present study shows that WT1 protein is developmentally expressed in the cytoplasm of human myoblasts from the 6 weeks of gestational age. This expression was maintained in the myotubes of developing muscles of the trunk, head, neck, and extremities, while it was down-regulated in fetal skeletal fibers from 20 weeks of gestational age as well as in adult normal skeletal muscle. Notably, WT1 immunostaining disappeared from rhabdomyomas, whereas it was strongly and diffusely re-expressed in all cases (27/27) of embryonal and alveolar rhabdomyosarcoma. The comparative evaluation of the immunohistochemical findings revealed that WT1 cytoplasmic expression in rhabdomyosarcoma may represent an ontogenetic reversal, and this nuclear transcription factor can also be considered an oncofetal protein which can be exploitable as an additional, highly sensitive immunomarker, together with desmin, myogenin and MyoD1, of this tumor. Moreover, our observations support the rationale for the use of WT1 protein-based target therapy in high risk rhabdomyosarcomas in children and adolescents.
authors: Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, AziendaOspedaliero-Universitaria "Policlinico-Vittorio Emanuele", Anatomic Pathology Section, School of Medicine, University of Catania, Catania, Italy. Electronic address: g.magro@unict.it.
authors: Gaetano
authors: G
authors: Magro
authors: Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, AziendaOspedaliero-Universitaria "Policlinico-Vittorio Emanuele", Anatomic Pathology Section, School of Medicine, University of Catania, Catania, Italy.
authors: Lucia
authors: L
authors: Salvatorelli
authors: Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, AziendaOspedaliero-Universitaria "Policlinico-Vittorio Emanuele", Anatomic Pathology Section, School of Medicine, University of Catania, Catania, Italy.
authors: Lidia
authors: L
authors: Puzzo
authors: Department of Biomedical and Biotechnological Sciences, Human Anatomy and Histology Section, School of Medicine, University of Catania, Catania, Italy.
authors: Giuseppe
authors: G
authors: Musumeci
authors: School of Biomedical Sciences, Etromapmacs Pole, Lesina, FG, Italy.
authors: Michele
authors: M
authors: Bisceglia
authors: Department of Biomedical and Biotechnological Sciences, Section of Physiology, School of Medicine, University of Catania, Catania, Italy.
authors: Rosalba
authors: R
authors: Parenti
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005333:Fetus / Q000196:embryology* / Q000473:pathology; D018507:Gene Expression Regulation, Developmental; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007223:Infant; D008297:Male; D019042:Muscle Neoplasms / Q000378:metabolism* / Q000473:pathology; D017570:MyoD Protein / Q000096:biosynthesis; D032448:Myoblasts, Skeletal / Q000378:metabolism* / Q000473:pathology; D012208:Rhabdomyosarcoma / Q000378:metabolism* / Q000473:pathology; D025721:WT1 Proteins / Q000096:biosynthesis*
chemical_list: D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein; D025721:WT1 Proteins; C583751:WT1 protein, human
keywords: Human embryo and fetus; Immunohistochemistry; Myogenesis; Rhabdomyosarcoma; WT1