title: Myofibrosarcoma of the head and neck in children.
abstract: We have identified a distinctive malignant soft tissue neoplasm that occurred in the head and neck region of six children. Histologically, these neoplasms presented an array of features ranging from low-grade spindle cell to high-grade fibrohistiocytic histologies and often had myoid characteristics. Ultrastructural and immunohistochemical studies indicated that they contained neoplastic myofibroblasts that were variably positive for vimentin (4 positive/4 tested), alpha-smooth muscle actin (4/5), muscle-specific actin (5/5), desmin (2/5), and v-src protein substrate p80/85 (4/5). Three patients died of rapidly progressive unresectable local disease, one died of metastatic and local disease, and two are alive 13 months and 8 years after wide resection. We conclude that these neoplasms form a distinctive subset of pediatric soft tissue sarcomas that display an aggressive clinical behavior, typically with local recurrence, and exhibit features of myofibroblastic differentiation.
authors.affiliation: Department of Pathology, University of Tennessee, Memphis, USA.
authors.forename: D M
authors.initials: DM
authors.lastname: Smith
authors.forename: H H
authors.initials: HH
authors.lastname: Mahmoud
authors.forename: J J
authors.initials: JJ
authors.lastname: Jenkins
authors.forename: B
authors.initials: B
authors.lastname: Rao
authors.forename: K P
authors.initials: KP
authors.lastname: Hopkins
authors.forename: D M
authors.initials: DM
authors.lastname: Parham
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005354:Fibrosarcoma / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D006258:Head and Neck Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D007890:Leiomyosarcoma / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D008297:Male; D009124:Muscle Proteins / Q000032:analysis
chemical_list: D009124:Muscle Proteins