Primary Ewing's sarcoma of the temporal bone: a rare oncological enigma

Abstract

Ewing's sarcoma is an uncommon neuroectodermal malignancy that rarely develops in the temporal bone. The early clinical recognition and definitive management of this condition pose unique challenges. This report presents a case of a 4-year-old girl with right ear pain, right post-aural swelling and progressively increasing right-sided facial nerve palsy for the past 6 months. High-resolution computed tomography (HRCT) temporal bone revealed an ill-defined soft tissue lytic lesion epi centred in the right mastoid extending to the external auditory canal and intracranially. Microscopic sections revealed round blue cells which were strongly positive for CD-99. The diagnosis of primary Ewing's sarcoma of the temporal bone was made. Considering the intracranial extension of the tumour, chemotherapy as the first line of treatment was initiated and 9 cycles have been administered resulting in marked regression of the tumour. A range of nonspecific symptoms, such as otalgia, otorrhea, sensorineural hearing loss, post aural mass, and facial nerve palsy, are indicative of the clinical manifestation of primary Ewing's sarcoma in the temporal bone. Because of how subtle these symptoms are, more clinical knowledge is required, especially in light of the small number of documented cases. When children and adolescents present with similar symptoms, clinicians should rule out Ewing's sarcoma even though it is a rare entity.