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Introduction: Chondrosarcoma is an unusual neoplasm in head and neck, characterized by the formation of cartilage. Material and methods: 27years old female with neurofibromatosis-1 presents a solid tumor in maxilla which has increased during last two months without symptoms. We took a biopsy and histology was mesenchymal chondrosarcoma, so we planned the surgical treatment. Results: We performed a left hemimaxilectomy type IIb (Brown and Shaw classification) and we reconstructed the defect with a fibula free flap. Histological exam confirmed the diagnosis and free margins. The patient received adjuvant radiotherapy and after 9months of follow up she presents good adaptation of the fibula free flap, an excellent oral mucosa metaplasia without recurrences. Conclusion: Chondrosarcoma represents 10–20% of all primary malignant bone tumours, but head and neck location is unusual ranging from 5% to 12% and only 0.1% of head and neck malignant tumours. It affects young people and the most frequent presentation is a painless swelling in the anterior maxilla. There are five different histological types: conventional, secondary, dedifferentiated, clear cell and mesenchymal. Our patient showed a mesenchymal chondrosarcoma which is always considered a high grade tumour. Metastasis appear in lungs but it not usually show up in neck lymph nodes. The most effective treatment is aggressive surgery with wide margins, also adjuvant treatment with radiotherapy and chemotherapy could be used in those patients but do not appear to have a significant effect on survival. Head and neck chondrosarcoma is a very rare neoplasm, It affects young people and presents different grades of malignancy depending on the histological type. Despite of the histology, the only really effective treatment is surgery, performing a wide margins resection and an appropriate reconstruction. [Copyright &y& Elsevier]

Abstract: Head and neck chondrosarcomas are rare and potentially lethal tumours, which are even more uncommon in the head and neck region. The diagnosis of chondrosarcoma is based on the histopathological pattern of malignant chondroid tissue proliferation. The grade of malignancy and the histological surgical margins are some of the most important prognostic factors for this group of tumour. In the present report we described two cases of chondrosarcoma affecting the maxilla, which presented different behaviours. The first patient, with a high-grade tumour, died of local recurrence and the second patient, with a low-grade tumour, is alive and free of disease 60 months after the treatment. These two cases emphasize the importance of histological tumour grade and appropriate treatment for the prognosis. [Copyright &y& Elsevier]