Extraskeletal Ewing's sarcoma of the head and neck region in pediatric patients: A case report and literature review

INTRODUCTION AND IMPORTANCE: Extraskeletal Ewing's sarcoma (EES) is a collection of malignant cells that appear small and round and occur mostly in pediatrics and adolescence. Head and neck EES tumors are considered rare and require multidisciplinary care to achieve ideal results in management. CASE PRESENTATION: A 14-year-old boy who complained of a mass protruding from the back of his neck which gradually increased in size in the last few months prior to the diagnosis. He was referred to a pediatric otolaryngology clinic with a one-year history of chronic painless nape swelling. Ultrasound prior to the referral was done and the findings revealed a well-defined rounded hypoechoic lesion with internal vascularity. MRI was done and the impression was a large subcutaneous, well defined enhancing soft tissue lesion which raised the suspicion of sarcoma. The multidisciplinary team decision was to go for complete resection with a free margin followed by chemoradiation postoperatively. No evidence of recurrence was detected throughout the follow-up. CLINICAL DISCUSSION: The literature review included ages of the pediatric group from 4 months up to 18-year-old. Clinical features are highly dependable on the size and site of the lesion. Complete resection of the tumor plays an important role in the local control and prognosis. CONCLUSION: We present a rare case of extraskeletal Ewing's sarcoma of the nape. Computed tomography and magnetic resonance imaging are frequently used as imaging modalities in evaluating and diagnosing EES. Management commonly includes surgery with adjuvant chemotherapy to decrease recurrence and prolong the survival rate.