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A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies
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Alveolar soft part sarcoma in a child - a case report.

V Bartoš, D Sejnová, A Skálová

    Klinicka Onkologie : Casopis Ceske a Slovenske Onkologicke Spolecnosti
    |October 25, 2023
    PubMed
    Summary

    Alveolar soft part sarcoma (ASPS) is a rare cancer. Complete surgical removal offers the best chance for a cure, especially in children, but long-term monitoring is crucial due to metastasis risk.

    Keywords:
    ASPSCR1:: TFE3alveolar soft part sarcoma

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    Area of Science:

    • Oncology
    • Pediatric Oncology
    • Genetics

    Background:

    • Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignancy with uncertain origins.
    • ASPS predominantly affects young individuals, with approximately 25% of diagnoses occurring in children.

    Observation:

    • An 11-year-old girl presented with a painless subcutaneous mass in her left elbow.
    • Imaging revealed a suspicious intramuscular soft-tissue mass, confirmed by biopsy as ASPS.
    • Histology showed an epithelioid-cell population with a pseudoalveolar pattern, TFE3 expression, and the ASPSCR1::TFE3 gene fusion.

    Findings:

    • Surgical excision of the ASPS lesion was performed.
    • A subsequent wide re-excision of the scar achieved complete tumor removal without microscopic residual disease.
    • The patient remains disease-free with no evidence of local recurrence or metastasis.

    Implications:

    • ASPS is an aggressive, chemoresistant neoplasm with a better prognosis in children than adults.
    • Early detection and complete surgical resection are critical for favorable outcomes.
    • Long-term follow-up is essential due to the potential for late metastases.