Abstract
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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Renard C, Ranchère-Vince D. Ewing/PNET sarcoma family of tumors: towards a new paradigm? Ann Pathol 2015; 35:86–97.
Asker S, Sayir F, Bulut G, Sunnetcioglu A, Ekin S, Yavuz A. Primitive neuroectodermal tumor/Ewing sarcoma presenting with pulmonary nodular lesions. Case Rep Oncol Med 2015; 2015:957239.
Miser JS, Kinsella TJ. Ewing’s sarcoma and the differentiated sarcomas. In: Pizzo PA, Poplack DG, editors. Principles and practices of pediatric oncology. [Chapter 31]. Philadelphia: JB Lippincott Company; 1989.
Hammar S, Bockus D, Remington F, Cooper L. The unusual spectrum of neuroendocrine lung neoplasms. Ultrastruct Pathol 1989; 13:515–560.
Takahashi D, Nagayama J, Nagatoshi Y, Inagaki J, Nishiyama K, Yokoyama R, et al. Primary Ewing’s sarcoma family tumors of the lung a case report and review of the literature. Jpn J Clin Oncol 2007; 37:874–877.
Dong M, Liu J, Song Z, Li X, Shi T, Wang D, et al. Primary multiple pulmonary primitive neuroectodermal tumor: case report and literature review. Medicine (Baltimore) 2015; 94:e1136.
Shet N, Stanescu L, Deutsch G. Primary extraosseous Ewing sarcoma of the lung: case report and literature review. Radiol Case Rep 2013; 8:832.
Lee YY, Kim DH, Lee JH, Choi JS, In KH, Oh YW, et al. Primary pulmonary Ewing’s sarcoma/primitive neuroectodermal tumor in a 67-year-old man. J Korean Med Sci 2007; 22(Suppl): S159–S163.
O’Keeffe F, Lorigan JG, Wallace S. Radiological features of extraskeletal Ewing sarcoma. Br J Radiol 1990; 63:456–460.
Milano GM, Cozza R, Ilari I, De Sio L, Boldrini R, Jenkner A, et al. High histologic and overall response to dose intensification of ifosfamide, carboplatin, and etoposide with cyclophosphamide, doxorubicin, and vincristine in patients with high-risk Ewing sarcoma family tumors. Cancer 2006; 106:1838–1845.
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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). https://doi.org/10.4103/ejb.ejb_48_16
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DOI: https://doi.org/10.4103/ejb.ejb_48_16