EWING’S SARCOMA OF THE MAXILLARY SINUS IN A CHILD: A CASE REPORT AND REVIEW OF LITTERATURE

Khaoula Hafidi*, Ilhame Toumir, Hasnae Taghzout, Zineb Alami, Touria Bouhafa and Khalid Hassouni

ABSTRACT

Ewing’s sarcoma is typically an aggressive, poorly differentiated tumor affecting children and young adults, it accounts for 4–6% of all primary bone tumors and facial primary localizations occur in only 1–4% of all cases, mostly in the mandible and calvaria. Paranasal sinus involvement is rare. Only 15 cases of extra-osseous Ewing sarcoma located in the nasal cavity and in the para-nasal sinus have been reported in the literature. The diagnosis of Ewing sarcoma is based on a complete imaging as well as on the anatomopathological analysis of the tumor tissues. It is characterized by dense clusters of small round cells in sheets, without tissue differentiation. It is a part of the family of neuroectodermal tumors, which have 11-22 translocation (or equivalent). Management must be multidisciplinary. The current treatment is based on the combination of chemotherapy and local treatment, at best surgical, sometimes radiotherapy. The two main prognostic factors are the existence of initial metastases and the response to chemotherapy. In the absence of treatment, the fatal course is usually rapid, with the appearance of pulmonary or bone metastasis. Advances in molecular biology can lead to hope for new treatments in the future. We report a new case of primitive Ewing sarcoma of the maxillary sinus in a 12-year-old child.

Keywords: Ewing’s sarcoma; Maxillary sinus; Chemotherapy; Radiotherapy.