Primary soft tissue Ewing's sarcoma of the maxillary sinus in elderly patients: presentation, management and prognosis

Abstract

Nonosseous or soft tissue Ewing's sarcoma is a rare form of Ewing's sarcoma/primitive neuroectodermal tumour that seldom affects the head and neck region. Involvement of the nose and paranasal sinuses is extremely uncommon, with only eight of such patients being reported to date, mostly affecting adolescents and young adults. To our knowledge, this study is the first comprehensive report of primary soft tissue Ewing's sarcoma involving the paranasal sinuses in an elderly patient who successfully completed treatment. We herein discuss the pathogenesis, management and factors affecting the prognosis of this rare group of tumours involving the nose and paranasal sinuses, in relation to the available literature.

Fig. 1

Photograph shows a huge swelling over the left side of the cheek. Note the narrowed palpebral fissure and difficulty in opening the left eye.

Fig. 2

(a & b) Axial and (c) contrast-enhanced coronal CT images of the nose and paranasal sinuses show a nonenhancing mass occupying the entire left maxillary antrum, resulting in its marked bulging in comparison to the opposite side. All the walls except the anterolateral wall are intact. The nasal cavity in the left is not involved.

Fig. 3

Photomicrographs of the biopsy specimen. (a) Sheets of small, round, uniform cells with indistinct cytoplasm, round-to-oval hyperchromatic nuclei, finely granular chromatin pattern and inconspicuous nucleoli, and few atypical mitoses are seen (Haematoxylin & eosin, × 400). (b) Immunohistochemistry was strongly and diffusely positive for CD99, with a distinct cytoplasmic membrane pattern.

Fig. 4

Photograph shows the excised specimen, measuring 6 cm × 5.5 cm × 4 cm.