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Primary pulmonary extraskeletal Ewing sarcoma/Primitive neuroectodermal tumor: Two case reports


Primary pulmonary extraskeletal Ewing sarcoma (EES)/ primitive neuroectodermal tumor is an extremely rare tumor with only few cases reported in the literature. In this study, we present two cases of primary pulmonary EES diagnosed and treated at our institution. The median age was 20 years (range: 19–21). Cough, dyspnea, and hemoptysis were the predominant features at presentation, associated with a large lung mass on imaging. Image-guided core needle biopsies were the diagnostic modalities for both patients. Initial histopathology showed malignant small round cell tumor, which has been confirmed by immunohistochemistry as EES. Both patients received neoadjuvant chemotherapy followed by surgery; postoperative pathology for the first patient showed 98% tumor necrosis, whereas the second patient’s pathology showed no evidence of residual tumor after complete surgical excision.


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Correspondence to Eman Sobh MD.

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This study was previously presented as oral abstract in Al-Azhar Grand Round and Alexandria Pediatric Pulmonology Conference.

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Sobh, E., El-Sheshtawy, W.H. & Anis, S.E. Primary pulmonary extraskeletal Ewing sarcoma/Primitive neuroectodermal tumor: Two case reports. Egypt J Bronchol 11, 161–164 (2017).

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  • Ewing sarcoma
  • extraskeletal Ewing sarcoma
  • lung primary
  • primitive neuroectodermal tumor