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.
MeSH terms
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Adolescent
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Base Sequence
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Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics
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Child, Preschool
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Humans
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Immunohistochemistry
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Intracellular Signaling Peptides and Proteins
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Male
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Molecular Sequence Data
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Mouth Neoplasms / genetics
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Mouth Neoplasms / pathology*
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Mouth Neoplasms / surgery
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Oncogene Proteins, Fusion / genetics
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Reverse Transcriptase Polymerase Chain Reaction
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Sarcoma, Alveolar Soft Part / genetics
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Sarcoma, Alveolar Soft Part / pathology*
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Sarcoma, Alveolar Soft Part / surgery
Substances
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ASPSCR1 protein, human
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Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
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Intracellular Signaling Peptides and Proteins
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Oncogene Proteins, Fusion
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TFE3 protein, human