Primary extraskeletal Ewing's sarcoma of the maxilla with intraorbital extension

Extraskeletal Ewing's sarcoma is often described as a tumour involving the soft tissues of the lower extremities and the paravertebral region. Primary Ewing's sarcoma of the cranium is extremely rare, with only 17 cases reported so far [24]. Involvement of the paranasal sinus is a very rare entity. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. Primary Ewings sarcoma rarely arises in the facial skeleton and only occasionally in the maxilla. To date, there have been 22 cases of maxillary Ewing's sarcoma reported in the English-language literature [25]. We report here a new case of Ewing's sarcoma localized to the maxillary sinus, nose and the orbit, successfully treated by surgery, local high dosage radiotherapy and systemic chemotherapy.