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.