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Bone sarcomas associated with Ollier's disease.

J Liu, P G Hudkins, R G Swee

    Cancer
    |April 1, 1987
    PubMed
    Summary

    Ollier's disease patients have a 30% risk of developing malignant bone neoplasms, primarily chondrosarcoma. Despite this risk, the prognosis for most patients with Ollier's disease is generally good.

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    Area of Science:

    • Orthopedics
    • Oncology
    • Pathology

    Background:

    • Ollier's disease is a rare congenital disorder characterized by enchondromatosis.
    • Malignant transformation is a known but infrequent complication of Ollier's disease.

    Purpose of the Study:

    • To determine the incidence and types of malignant bone neoplasms in patients with Ollier's disease.
    • To assess the prognosis associated with malignant transformation in Ollier's disease.

    Main Methods:

    • Retrospective review of 55 patients diagnosed with Ollier's disease.
    • Analysis of medical records for the development of bone neoplasms and their histological types.
    • Evaluation of patient survival and treatment outcomes.

    Main Results:

    • 16 out of 55 patients (approximately 29%) developed malignant bone neoplasms.
    • The most common malignancy was chondrosarcoma (12 cases), including two dedifferentiated chondrosarcomas.
    • Other malignancies included one chordoma and one osteosarcoma.
    • One patient developed chondrosarcoma in two separate bones.
    • Five of the 16 patients with malignant neoplasms survived for over 13 years post-treatment.

    Conclusions:

    • Approximately 30% of Ollier's disease patients are at risk for developing malignant bone neoplasms.
    • Chondrosarcoma is the most probable malignant neoplasm to arise in patients with Ollier's disease.
    • The overall prognosis for patients with Ollier's disease and subsequent malignant transformation appears to be favorable for many, with long-term survival achievable.

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