Chondroid chordoma in an atypical location

Chondroid chordoma is an extremely rare tumour with an annual incidence of around 0.1 cases per 100,000population. Involvement of the thoracic vertebrae may be present in 2-5% of cases. Definitive diagnosis usually requires a suitable distinction between this and other mesenchymal tumours such as chondrosarcomas, so immunohistochemical analysis is virtually mandatory. In spite of its slow-growing nature, chondroid chordoma tends to relapse, and it may eventually become malignant, often jeopardising the patient's prognosis. Although surgery remains the main therapeutic approach, research into the molecular and genetic aspects of this tumour is ongoing. These new advances are likely to improve future oncology therapies by complementing surgery and radiotherapy, changing the currently poor prognosis. We report the case of a patient with a chondroid chordoma involving the thoracic vertebrae and pleural cavity, and the treatment performed.