Primary Extraskeletal Ewings Sarcoma of Ala of Nose . 1

Extra skeletal Ewing's sarcoma is often described as a tumour involving the soft tissues of the lower extremities and the para vertebral region. Ewing's sarcoma presents a rare tumour of the head and neck, and even rarer in the nasal cavity and/or paranasal sinuses. Primary Ewing’s sarcoma of the ala of the nose has not been commonly described previously in the English literature. We present a case of primary Ewing's sarcoma of the nose in a 23-year-old male, presented as a fleshy mass in the ala of the left side of the nose with symptoms of nasal obstruction. The obstructing lesion was excised and microscopy showed a neoplasm composed of comparatively uniform undifferentiated cells forming solid nests. The cytoplasm of the cells was clear but poorly demarcated, partly vacuolated and contained much glycogen. The cells expressed the MIC2 gene (using the CD99 marker). Managed with wide local excision and all resection margins are microscopically free of tumour margins. Post-operative adjuvant chemotherapy was started with High-Risk protocol (vincristine, doxorubicin, cyclophosphamide and mesna alternating every 3 weeks combined ifosfamide and etoposide) and is under follow up. An extensive review of literature, to the best of our knowledge, did not reveal many cases of EES of ala of nose.