Primary Ewing Sarcoma of the Sinonasal Tract- A Case Report

Abstract

A 21-year-old male presented with swelling in right medial canthus for 5 months. The swelling was soft to firm, nontender and immobile, measuring 2.5 cms x 1.5 cms. On local examination surface was nodular on medial part without redness, scar or puncta. The nodule was seen extending superiorly up to the level of medial end of the right eyebrow, inferiorly to 5 mm below the level of right lower eyelid, medially close to the midpoint of the nasion and laterally upto the medial canthus of right eye till the right eyebrow [Figure 1]. With a preliminary diagnosis of dacryocystocele, further investigations were ordered. Ewing sarcoma is a small round cell malignant neoplasm, considered to be of neuroectodermal derivation. It is relatively uncommon accounting for 6%-8% of primary malignant bone tumours and the second most common sarcoma in bone and soft tissue in children. 1 James Ewing reported a round cell neoplasm in the radius of a 14-year-old girl in 1921. Angervall and Enzinger in 1975 described the first Ewing's Sarcomas arising in soft tissue (extraskeletal ES). Seemayer et al and Askin et al described malignant small cell tumours arising in the soft tissues having histologic features similar to peripheral neuroectodermal tumours (PNET), but with unique clinico-pathologic profile. With the advent of immunohistochemical, cytogenetic and molecular genetic techniques, these tumours are universally regarded to represent ends of a morphologic spectrum known as ES/PNET family of tumours. 2 Ewing Sarcoma (ES) has both skeletal and extraskeletal forms. 3 The extraskeletal form usually occurs in the soft tissues of lower extremities, paravertebral tissues, chest wall and retroperitoneum. 4 Primary Ewing Sarcoma of the head and neck region is extremely rare and accounts for only 4%-9% of all Ewing Sarcomas with a slight male preponderance. 2,5 Extraskeletal Ewing Sarcoma in the sinonasal tract is so rare that only a few case reports have been published in the world literature. 5