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

Abstract

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. Keywords Ewing's sarcoma Paranasal sinusEwing's sarcoma is a malignant neoplasm of uncertain histogenesis, most commonly arising in the skeleton. The head and neck are unusual sites for primary Ewing's sarcoma. Primary Ewing's sarcoma of the paranasal sinus (PNS) is exceptionally rare and most cases are described in the maxillary sinus [16]. Of the 14 cases that we are aware of reported in the literature, the maxillary sinus (7 cases) is the most common site followed by the ethmoid sinus (2 cases ) and the nasal fossa (5 cases ) [1][2][3][4][5][6][7][8][9][10].

Ewing′s sarcoma of maxilla: A rare case report

“…When the jaws are involved it frequently involves the mandible than maxilla. [ 5 6 7 8 9 ] Out of 9 cases analyzed with Ewing's sarcoma of maxilla, 6 were seen in males and 3 in females with a male to female ratio of 2:1 [ Figure 9 ]. The median age of the patient was 17 years.…”

“…The majority of patients had a average tumor size of 6 × 4 cm. [ 1 6 10 11 12 13 14 15 ] [ Figure 10 ]. Out of these cases, one case presented with a history of trauma.…”