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[Head and neck alveolar soft-part sarcoma: a review in diagnosis and treatment]

H Xiao, H Yang, D Lv

    Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi = Journal of Clinical Otorhinolaryngology Head and Neck Surgery
    |June 6, 2018
    PubMed

    Insights

    Head and neck alveolar soft part sarcoma (ASPS) is a rare pediatric cancer. Understanding its genetic cause, the ASPL-TFE3 fusion gene, is key to developing new molecular therapies for this aggressive disease.

    Area of Science:

    • Oncology
    • Pediatric Oncology
    • Sarcoma Research

    Context:

    • Alveolar soft part sarcoma (ASPS) is a rare and aggressive cancer.
    • This distinctive sarcoma primarily affects infants and children.
    • Head and neck ASPS presents unique diagnostic and therapeutic challenges.

    Purpose:

    • To review the current diagnosis and treatment strategies for head and neck alveolar soft part sarcoma (ASPS).
    • To highlight the molecular underpinnings of ASPS, specifically the ASPL-TFE3 fusion gene.
    • To discuss the potential of novel molecularly targeted therapies.

    Summary:

    • Alveolar soft part sarcoma (ASPS) is characterized by a rapid clinical course and poor prognosis, often involving late metastases.
    • The genetic hallmark of ASPS is the ASPL-TFE3 fusion gene, identified as the causative molecular driver.
    • Emerging molecularly targeted therapies show promise for altering the treatment paradigm for primary ASPS.

    Impact:

    • Provides a comprehensive overview of head and neck ASPS diagnosis and management.
    • Emphasizes the critical role of molecular diagnostics in understanding ASPS.
    • Suggests a future direction for ASPS treatment through targeted molecular therapies.

    Keywords:
    alveolar soft-part sarcomadiagnosishead and neck neoplasmstreatment

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