Sarcome d'ewing du rocher

Introduction : Primary cranial Ewing's sarcoma is rare. This tumor was first described in 1921, since then, only a few cases have been reported in the literature. Aim: the aim of this study is to report the clinical and therapeutic aspects of primary cranial Ewing's sarcoma. Method: We report a case of Ewing's sarcoma of the temporal bone in a 16 months child diagnosed and treated in our department of ENT of Tunis. Result: the diagnostic of Ewing's sarcoma was made after bleeding ear. Histological confirmation was made following biopsy. After neoadjuvant chemotherapy, the tumor was surgically excised and the patient underwent chemotherapy. Neither recurrence nor distant metastasis was noted a 34 months. Conclusion: Although the prognosis of Ewing's sarcoma in general is often poor because of early metastasis to the lungs and/or to other bones, the same tumor occurring in the cranium can often be successfully managed by intensive therapy with radical excision and radiochemotherapy.