Ewing’s sarcoma (ES) is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells. Primary ES commonly occurs in early childhood or adolescence. It may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is a case report of sinonasal ES in a 13-year-old female patient who presented with a 7-month history of right nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss. Clinical examination revealed a right nasal mass pushing the septum to the left side and extending to the nasopharynx. Endoscopic biopsy and histopathological analysis showed a small blue cell tumor suggestive of ES. The patient was treated with surgery, radiotherapy, and chemotherapy. After a follow-up of 5 years, the patient remains recurrence-free with excellent functional status and quality of life.
Ewing’s sarcoma (ES) is a highly malignant and rare tumor that subdivided into skeletal and extraskeletal entities [
On March 2010, a 13-year-old female patient referred to the ENT clinic complaining of right sided nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss for 7-month duration. There was no history of trauma, anorexia, or weight loss.
Clinical examination revealed a right sided nasal mass pushing the septum to the left side and extending to the nasopharynx. On throat examination, the soft palate was pushed down by the nasopharyngeal mass. Otoscopy showed dullness and retraction of tympanic membrane bilaterally. Cranial nerves examination was normal. No cervical lymph nodes were palpable. The results of hematological and biochemical investigations were within normal limits.
On radiological evaluation, CT scan revealed an opacification of the right nasal cavity, maxillary, ethmoidal, sphenoid, and frontal sinuses with bone remodeling of the septum to the left side (Figure
Computed tomography (CT) scan showing a mass causing an opacification of the right nasal cavity, ethmoid and maxillary sinuses.
Microscopic analysis showing sheets of small round blue cells.
Immunohistochemistry of tumour sample showing CD99 positivity.
ES is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells [
ES is a rare disease that accounts for only 4% to 6% of all primary bone tumors [
Clinical manifestations of sinonasal ES include enlarging mass, nasal obstruction, rhinorrhea, and epistaxis [
The prognosis depends on the site of the primary tumor, the presence of distant metastasis at presentation, and the age of the patient [
Successful treatment of ES includes a multidisciplinary approach with surgery followed by adjuvant radiotherapy and chemotherapy [
Ewing’s sarcoma rarely affects the sinonasal tract. Diagnosis of the disease is challenging hence it requires histopathological examination, immunohistochemical studies, and cytogenetic studies. Treatment includes a multidisciplinary approach with surgery followed by chemotherapy and radiotherapy.
The authors declare that there are no conflicts of interest regarding the publication of this paper.