title: Pediatric sclerosing rhabdomyosarcoma.
abstract: Sclerosing rhabdomyosarcoma, a particular phenotypic variant of rhabdomyosarcoma initially described in the adult population, has emerged as a potential pitfall in the evaluation of pediatric sarcomas. Because of its densely hyalinized collagenous matrix and its occasional expression of a pseudovascular pattern of growth, sclerosing rhabdomyosarcoma has been at times misdiagnosed as chondrosarcoma, osteosarcoma, or angiosarcoma. We describe 3 pediatric patients with sclerosing rhabdomyosarcoma and provide a detailed description of its distinguishing pathologic features. Awareness about this rhabdomyosarcoma variant and careful immunophenotypical evaluation are necessary to establish the correct diagnosis. Although no specific genetic aberrations have been recognized, yet the cytogenetic findings in 2 tumors of this series suggest a link with embryonal rhabdomyosarcoma. It is likely that further genotyping will result in better nosologic delineation of sclerosing rhabdomyosarcoma and that it will uncover pathogenetically and prognostically relevant genes.
authors.affiliation: Department of Pathology, Yale-New Haven Hospital, Yale School of Medicine, New Haven, Connecticut 06520, USA. eduardo.zambrano@yale.edu
authors.forename: Eduardo
authors.initials: E
authors.lastname: Zambrano
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Pérez-Atayde
authors.forename: William
authors.initials: W
authors.lastname: Ahrens
authors.forename: Miguel
authors.initials: M
authors.lastname: Reyes-Múgica
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D012208:Rhabdomyosarcoma / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D012598:Sclerosis / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor