Ewing sarcoma (ES), often reported also as primitive neuroectodermal tumour (PNET), is an uncommon neuroectodermal malignant neoplasm, accounting for 6–8 % of primary malignant bone tumours, and represents the second most common bone tumour in children, adolescents, and young adults [1]; usually involves the long bones, the pelvis, and the ribs [2]. From a pathophysiological perspective, ES is a tumour triggered by a reciprocal translocation t(11;22) (q24;q12) involving EWSR1 and the transcription factor FLI1; the N-terminal region of the EWSR1 protein comprises a strong activation domain, causing aberrant transcription of numerous genes [3]. In addition, EWS-containing fusion proteins are known to regulate epigenetic determination, splicing, and metabolism of cells.

Extra-skeletal ESs are relatively rare accounting for about 1 % of soft tissue sarcomas: the most frequent extraosseous localizations include the chest wall, paravertebral and gluteus muscles, and retroperitoneal space, but also other rarer localizations have been described [4]. Only sporadic cases of primary pulmonary ES (PES) have been reported in the scientific literature, mostly in the form of single case reports; it has been estimated that less than 40 cases have been published since 1989 when Hammar et al. described the first case [5], [6]. Therefore, few data are currently available regarding the clinical, pathological, and molecular features of these tumours. We performed a systematic review of the recent literature with the aim to describe the clinical manifestations, the pathological and diagnostic features, as well as the therapeutic approaches and the respective prognostic results obtained in patients affected by PES.