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  • Title: [Parapharyngeal chondrosarcoma and Ollier's disease: a case report and review of the literature].
    Author: Nengsu A, Bernat I, Brocheriou-Spelle I, Martin-Duverneuil N, Lamas G, Soudant J, Tankéré F.
    Journal: Ann Otolaryngol Chir Cervicofac; 2002 Nov; 119(5):287-92. PubMed ID: 12464854.
    Abstract:
    OBJECTIVES: Ollier's disease is a constitutional pathology of unknown etiology. It is characterized by bone dysplasia generating numerous enchondromas. The malignant degeneration of this dysplasia is well known. The aim of this article is to study the diagnostic, therapeutic and prognostic characteristics of these lesions. MATERIAL AND METHODS: We report a case of parapharyngeal chondrosarcoma extended to the base of the skull in a patient with Ollier's disease. The treatment was a surgical removal by a cervicotransoral incision combined with a preauricular incision and with a mastoidectomy. It was therefore possible to control the skull base, the parapharyngeal space, the infratemporal fossa and the major neurovascular structures. The removal of the lesion was completed at the level of the clivus and sphenoid with optics (30 and 70 degrees ). We discuss this treatment and the follow up on the bases of literature data. RESULTS: The neoplastic degeneration of enchondromas is estimated between 25 to 50% of cases. The most frequent location is the pelvic bones. Chondrosarcomas are slow growing tumors and their metastatic potential is less significant as we note it in our case report. Their diagnostic is essentially based on histological criteria's and their treatment is surgical. CONCLUSION: Chondrosarcomas of the ENT area occurring with Ollier's disease is rare. Their prognostic is good if the surgical treatment is well done.
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