Primary Ewing sarcoma of the lung: A systematic review of the recent literature

Primary pulmonary Ewing sarcoma (PES) is a rare malignancy with only sporadic cases reported in the scientific literature. We performed a systematic review of the cases published in the last decade on PubMed, with the aim to describe the clinical, pathological, therapeutic, and prognostic data of PES. Forty-two articles reporting on 50 cases have been reviewed. Globally, 60 % of the patients were males, and the mean age at diagnosis was 30.5 years, with only a few cases diagnosed after 50 years of age. The most common clinical manifestations at diagnosis were dyspnea, cough and chest pain. The most common immunohistochemistry findings were staining for CD99 and (less frequently) for vimentin, and no staining for TTF-1, cytokeratin, desmin and S-100. ESWR1-FL1 translocation was tested in less than half of the cases. The disease was often locally advanced, treated generally with multidisciplinary treatment combining surgery, chemotherapy and radiation therapy. Among patients with follow-up data, approximately 40 % were dead at the time of publication, with the median survival being 11.5 months. Among those who were alive, only 8.3 % was free from disease at 48 months from diagnosis.