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[Sacral chordoma, a rare tumor].

J Closset, F Mariscal, M Herin

    Acta Chirurgica Belgica
    |May 1, 1986
    PubMed
    Summary

    Chordoma is a rare, slow-growing malignant tumor. Diagnosis involves imaging and biopsy, with surgery as the primary treatment, though complete resection is challenging and recurrence is common.

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    Area of Science:

    • Oncology
    • Surgical Pathology

    Background:

    • Chordoma is a rare malignant tumor originating from notochordal remnants.
    • It is characterized by slow growth and insidious onset of symptoms due to compression of surrounding structures.

    Purpose of the Study:

    • To describe a case of chordoma.
    • To review the literature on chordoma, focusing on diagnosis, treatment, and outcomes.

    Main Methods:

    • Literature review.
    • Diagnostic imaging including standard radiography, tomography, echography, and computed tomography.
    • Biopsy for definitive diagnosis.
    • Surgical resection using a combined abdominal and trans-sacral approach.

    Main Results:

    • Chordoma is a rare malignant tumor with slow growth.
    • Diagnosis is aided by various imaging modalities and confirmed by biopsy.
    • Surgical resection is the primary treatment, but complete removal is often limited by functional preservation.
    • Radiation therapy is frequently used after subtotal resection.
    • Local recurrence is common, while metastasis is rare.
    • The 10-year survival rate is approximately 10%.

    Conclusions:

    • Chordoma presents a significant challenge in surgical management due to the difficulty of achieving complete resection while preserving function.
    • High rates of local recurrence necessitate further investigation into optimal treatment strategies.
    • The prognosis for chordoma remains poor, with a high mortality rate within ten years.

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