title: Oral alveolar soft part sarcoma in childhood and adolescence: report of two cases and review of literature.
abstract: Alveolar soft part sarcoma (ASPS) constitutes a rare soft tissue malignant neoplasm comprising less than 1 % of all soft tissue sarcomas. ASPS demonstrates a strong predilection for adolescents and young adults, with a female predominance reported. The head and neck region is the most commonly affected region in pediatric patients with the tongue and orbit affected most commonly. Herein we present the clinical, radiographic, histopathologic, immunohistochemical and molecular features of two examples of ASPS affecting the oral cavity of 4 and 13 year-old boys, along with a focused review of the literature on intraoral ASPS in pediatric patients.
authors.affiliation: Department of Oral Pathology and Medicine, School of Dentistry, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Prokopios P
authors.initials: PP
authors.lastname: Argyris
authors.forename: Robyn C
authors.initials: RC
authors.lastname: Reed
authors.forename: J Carlos
authors.initials: JC
authors.lastname: Manivel
authors.forename: Dolores
authors.initials: D
authors.lastname: Lopez-Terrada
authors.forename: Jared
authors.initials: J
authors.lastname: Jakacky
authors.forename: Zuzan
authors.initials: Z
authors.lastname: Cayci
authors.forename: Konstantinos I
authors.initials: KI
authors.lastname: Tosios
authors.forename: Stefan E
authors.initials: SE
authors.lastname: Pambuccian
authors.forename: Lester D R
authors.initials: LD
authors.lastname: Thompson
authors.forename: Ioannis G
authors.initials: IG
authors.lastname: Koutlas
mesh_terms: D000293:Adolescent; D001483:Base Sequence; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics; D002675:Child, Preschool; D006801:Humans; D007150:Immunohistochemistry; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008969:Molecular Sequence Data; D009062:Mouth Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018234:Sarcoma, Alveolar Soft Part / Q000235:genetics / Q000473:pathology* / Q000601:surgery
chemical_list: C422743:ASPSCR1 protein, human; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D047908:Intracellular Signaling Peptides and Proteins; D015514:Oncogene Proteins, Fusion; C069203:TFE3 protein, human