title: [Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component].
abstract: An exceptional case of malignant peripheral nerve sheath tumor with striated muscle differentiation and glandular component is reported, in a 52-year old man. This tumor measured 8 cm in diameter, and was localized in the chest wall, infiltrating the skeletal muscle. The mesenchymal portion of the tumor was composed mostly of spindle cells arranged in interlacing fascicles. Between these fascicles, there were large cells with abundant eosinophilic cytoplasm and clear elongated nucleus. Immunohistochemical study demonstrated cytokeratin, EMA and CEA expression in the glandular component and S100 protein expression in the major portion of the mesenchymal component. The large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, alpha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resection. Histological and immunohistochemical features were identical. We think that positivity of neoplastic striated muscle cells with alpha-SM actin reflects an early differentiation phase of these cells.
authors.affiliation: Division de Pathologie, Institut Central des Hôpitaux Valaisans, avenue du Grand-Champsée 86, 1951 Sion, Suisse.
authors.forename: V
authors.initials: V
authors.lastname: Karpuz
authors.forename: N
authors.initials: N
authors.lastname: Letovanec
authors.forename: A
authors.initials: A
authors.lastname: Von Hochstetter
authors.forename: F
authors.initials: F
authors.lastname: Joris
mesh_terms: D002272:Carcinoembryonic Antigen / Q000032:analysis; D050701:E2F6 Transcription Factor; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009361:Neoplasm Invasiveness; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000601:surgery; D010524:Peripheral Nervous System Neoplasms / Q000473:pathology* / Q000601:surgery; D012097:Repressor Proteins / Q000032:analysis; D012208:Rhabdomyosarcoma / Q000473:pathology* / Q000601:surgery; D009418:S100 Proteins / Q000032:analysis; D013899:Thoracic Neoplasms / Q000473:pathology / Q000601:surgery; D014157:Transcription Factors / Q000032:analysis
chemical_list: D002272:Carcinoembryonic Antigen; D050701:E2F6 Transcription Factor; C491744:E2F6 protein, human; D012097:Repressor Proteins; D009418:S100 Proteins; D014157:Transcription Factors