title: [Infantile rhabdomyofibrosarcoma. An aggressive tumor in the spectrum of spindle cell tumors in childhood].
abstract: We report a case of an intrathoracic, extrapleural, infantile rhabdomyofibrosarcoma in a 4-year-old boy. Histologically, the primary lesion showed extensive hyalinization and stroma sclerosis and was composed of relatively uniform spindle-shaped, at least focally rather polygonal tumour cells with scattered intracytoplasmatic globoid inclusions. Although chemo- and radiotherapy was given postoperatively, local recurrences and metastases in the lung and thymus have developed; the patient died of tumour disease 3 years later. Recurrences and metastases showed features of tumour progression with higher cellularity and increased mitotic activity. Immunohistochemically, the tumour cells stained strongly positive for vimentin, desmin, and muscle-specific actin, and at least focally for MyoD1; the tumour did not stain for alpha-smooth muscle actin, neural and epithelial markers, or CD34 and CD31. The differential diagnosis of these aggressive tumours in the spectrum of spindle-cell lesions in early childhood is discussed.
authors.affiliation: Institut für Pathologie, Friedrich-Schiller-Universität, Jena.
authors.forename: T
authors.initials: T
authors.lastname: Mentzel
authors.forename: H J
authors.initials: HJ
authors.lastname: Mentzel
authors.forename: D
authors.initials: D
authors.lastname: Katenkamp
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D007124:Immunoenzyme Techniques; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D008938:Mitosis / Q000502:physiology; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000628:therapy; D018233:Rhabdomyosarcoma, Embryonal / Q000473:pathology* / Q000628:therapy; D013899:Thoracic Neoplasms / Q000473:pathology* / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor