Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature.
ABSTRACT Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities. We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma. Histopathological examination showed a small round cell tumor. Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma. Cytogenetic analysis revealed a high level of aneuploidy in the tumor cells, with double-minutes and additional chromosomal structural aberrations. The patient is responding well to chemotherapy.
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ABSTRACT: Introduction: Rhabdomyosarcoma is a malignant neoplasm with origin from voluntary muscles and causes approximately 50% of all tumors of soft part in children. The embryonal subtype is the most common in the head and neck region, its location in the middle ears and mastoid is uncommon and of the worst prognosis due to the high incidence of intracranial extension. the signs and symptoms depend on the location of the tumor. Objective: The objective of this case report was to carry out a brief review on the Rhabdomyosarcoma and Gradenigo's Syndrome association and clarify the importance of the differential diagnosis in this kind of pathology. Case Report: In this work we report the case of a five-year-old boy with diagnosis of temporal bone Rhabdomyosarcoma that presented with a case of Gradenigo's Syndrome (abducens paralysis, otorrhea/otalgia, retroorbitary pain) and facial nerve paralysis.
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ABSTRACT: Introdução: Rabdomiossarcoma é uma neoplasia maligna com origem em músculos estriados e é responsável por aproximadamente 50% de todos os tumores de partes moles em crianças. O subtipo embrionário é o mais comum na região da cabeça e pescoço, sua localização na orelha média e mastoide é rara e de pior prognóstico devido à alta incidência de extensão intracraniana.Os sinais e sintomas dependem da localização do tumor. Objetivo: O objetivo desse relato de caso foi realizar uma breve revisão sobre a associação Rabdomiossarcoma e Síndrome de Gradenigo e elucidar a importância do diagnóstico diferencial nesse tipo de patologia. Relato do Caso: Nesse trabalho é relatado o caso de um menino de cinco anos de idade com o diagnóstico de Rabdomiossarcoma de osso temporal que se apresentou com o quadro de Síndrome de Gradenigo (paralisia do abducente, otorreia/otalgia, dor retro-orbitária) e paralisia do nervo facial.
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ABSTRACT: Rhabdomyosarcomas are a diverse group of malignant mesenchymal neoplasms exhibiting variable levels of differentiation toward skeletal myocytes. Neoplastic cells may resemble relatively undifferentiated myoblasts, satellite cells, or more differentiated elongated spindle cells and multicellular myotubes. In veterinary medicine, classification into subtypes and variants is based on an outdated system derived from human pathology and is solely based on histologic characteristics. In contrast, classification of human rhabdomyosarcoma is based on histologic, immunohistochemical, and molecular diagnostic techniques, and subclassification has clinical and prognostic relevance. Relevance of tumor subtyping has not been established in veterinary medicine. Recent discoveries of components of the molecular pathogenesis and genomes of human rhabdomyosarcomas have led to new diagnostic techniques and revisions of the human classification system. The current classification system in veterinary medicine is reviewed in light of these changes. Diagnosis of rhabdomyosarcoma using histopathology, electron microscopy, and the clinical aspects of human and canine rhabdomyosarcomas is compared. The clinical features and biologic behavior of canine rhabdomyosarcomas are compared with canine soft tissue sarcomas.Veterinary Pathology 02/2013; 50(5). DOI:10.1177/0300985813476069 · 1.87 Impact Factor