We report on a patient who presented in ENT clinic with diplopia accompanied by headache and vomiting. Imaging studies revealed a large expansive heterogeneous mass encroaching on the infrasellar region and cervical spine. Post-intravenous contrast administration revealed a lesion with high enhancement and involvement of the sphenoidal sinus with retroclival extension. Biopsy confirmed the presence of a chondroid chordoma. Treatment included radiation therapy and the patient is currently receiving palliative and supportive care.

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Synovial sarcoma (SS) represents about 10% of all soft tissue sarcomas. It is believed that its origin would be found in cells that are related neither to ultrastructural nor to histological features of the synovial tissue. Head and neck is very rarely affected, with the lower extremities being most frequent. Complete resection with or without radiotherapy and chemotherapy is currently considered the best available therapy. This time we present the case of a patient with SS located in the infratemporal fossa, its diagnosis, treatment and evolution. According to our knowledge it is the first reported case in South America.