Extraosseous Ewing's Sarcoma of the Maxillary Sinus: A Very Rare Entity

Abstract

AbstractEwing's sarcoma is rare sarcoma of bone and soft tissue that uncommonly involve the head and neck in both bone and soft tissue locations. The term Ewing's sarcoma family of tumors comprises both Ewing's sarcoma and peripheral neuroectodermal tumor. It was in 1975, Angervall and Enzinger described the Ewing's sarcoma arising in soft tissues (extraskeletal). The principal sites of extraskeletal Ewing's sarcoma are the paravertebral region and chest wall, generally in close association with vertebrae or the ribs. These tumors may also arise in the soft tissues of the lower extremities and rarely in the pelvic region and hip regions. Superficially located cases do occur but are rare. Here, we present a case of Ewing's sarcoma involving the maxillary sinus region. A 12-year-old boy reported to our department with a swelling on the right side of middle third of the face for 15 days. Intraorally, there was only obliteration of the labial vestibule. On histopathological examination, a provisional diagnosis of malignant round cell tumor was given. Immunohistochemistry with a panel of markers was done to come to a definitive diagnosis. CD99 positivity confirmed the diagnosis of Ewing's sarcoma.