Abstract
Extraskeletal Ewing sarcoma (EES) is a tumour of rare variant of the Ewing sarcoma family of tumours. This family of tumours can have different features; however, these tumours are categorised on the basis of genetic translocation, specific molecular and immunohistochemical features. EES is seen commonly affecting young adults with poor prognosis and high mortality rates. It can be detected in various locations making its diagnosis more difficult. It can present with varied imaging features, often non-specific. However, imaging plays a vital role in the primary tumour assessment, local staging, preoperative management and surveillance. Management involves surgery with chemotherapy. Long-term prognosis in cases of metastatic disease is very poor. In literature, only three cases of axillary EES have been reported so far. Here, we report the fourth case of large EES originating in the left axillary region in a woman in her 20s. The patient was given neoadjuvant chemotherapy; however, the size of the tumour increased, which was later surgically treated with complete excision of the tumour. Unfortunately, the tumour metastasised to the lung for which the patient was irradiated. Afterwards, the patient presented to the emergency room with respiratory distress for which she was on ventilator support; sadly, the patient died after 1 week.