{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T05:38:04Z","timestamp":1781156284107,"version":"3.54.1"},"reference-count":33,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T00:00:00Z","timestamp":1609977600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p><jats:bold>Background:<\/jats:bold> Ewing sarcoma is the second most common malignant bone tumor in children, but it rarely originates from extra-skeletal sites. The commonly involved sites of soft tissue include paravertebral spaces, lower extremities, the pelvis, head, and neck, while primary extra-skeletal Ewing sarcoma (EES) located in the genitals is extremely rare.<\/jats:p><jats:p><jats:bold>Case Presentation:<\/jats:bold> We report a young patient who presented to our hospital with a painful erection of the penis and limited motion of the left hip. Magnetic resonance imaging showed a hyperintense mass with invasion of adjacent tissue in the penis and a heterogeneously high signal lesion in the left proximal femur. <jats:sup>18<\/jats:sup>F-fluorodeoxyglucose positron-emission tomography detected widespread metastatic lesions in the bilateral lung and multiple skeletons. An incisional biopsy of the penis was performed; the histopathological findings and EWS gene translocation identified by molecular analysis confirmed the diagnosis of Ewing sarcoma. Subsequently, the punch-biopsy specimen from the left femur showed undifferentiated small round cells, a finding consistent with the microscopic presence of Ewing sarcoma metastasis. However, after the first course of multiagent chemotherapy, the penile mass did not obtain stabilization but instead grew progressively with surface ulceration and multidrug resistant bacteria infection. Despite receiving antibiotics and maximal supportive therapy, the patient died from sepsis and lung metastasis complications in the intensive care unit 2 months later.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> This case indicates that although EES as a subtype of Ewing sarcoma is rare, it can occur virtually in any soft tissue site, even in the genitals. Therefore, clinicians need to distinguish this entity from other soft tissue sarcomas with rapid progression since early diagnosis and timely treatment of EES are pivotal for a favorable prognosis.<\/jats:p>","DOI":"10.3389\/fped.2020.591257","type":"journal-article","created":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T06:09:36Z","timestamp":1609999776000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Case Report: Primary Ewing Sarcoma of the Penis With Multiple Metastases"],"prefix":"10.3389","volume":"8","author":[{"given":"Chuanxi","family":"Zheng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Luo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongying","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chongqi","family":"Tu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Min","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,1,7]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1111\/his.12281","article-title":"Round cell sarcomas beyond Ewing: emerging entities","volume":"64","author":"Antonescu","year":"2014","journal-title":"Histopathology"},{"key":"B2","volume-title":"WHO Classification of Tumours of Soft Tissue and Bone","year":"2013"},{"key":"B3","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-319-01748-8","volume-title":"Atlas of Musculoskeletal Tumors and Tumorlike Lesions: The Rizzoli Case Archive","author":"Picci","year":"2014"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001622","article-title":"Pediatric extraskeletal Ewing sarcoma originating in the heart: a case report and review of the literature","author":"Buffoni","year":"2019","journal-title":"J Pediatr Hematol Oncol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"18","DOI":"10.7812\/TPP\/18-255","article-title":"Extraskeletal Ewing sarcoma of the jejunum: a case report","volume":"23","author":"Cantu","year":"2019","journal-title":"Perm J"},{"key":"B6","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1007\/s00404-011-2011-x","article-title":"Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature","volume":"285","author":"Kelling","year":"2012","journal-title":"Arch Gynecol Obstet"},{"key":"B7","doi-asserted-by":"publisher","first-page":"e574","DOI":"10.3747\/co.26.5001","article-title":"Primary Ewing sarcoma of the larynx with distant metastasis: a case report and review of the literature","volume":"26","author":"Maroun","year":"2019","journal-title":"Curr Oncol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1177\/1066896912454565","article-title":"Primary Ewing's sarcoma\/primitive neuroectodermal tumor (ES\/PNET) of the penis: a report of an extraordinarily unusual site and a literature review of extraskeletal Ewing's sarcoma","volume":"21","author":"Ma","year":"2013","journal-title":"Int J Surg Pathol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"556.e1","DOI":"10.1016\/j.jpurol.2019.06.022","article-title":"Ewing sarcoma\/primitive neuro-ectodermal tumor of the urogenital system in children: a retrospective observational case series","volume":"15","author":"He","year":"2019","journal-title":"J Pediatr Urol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1273","DOI":"10.1111\/jgh.14638","article-title":"Gastrointestinal: multi-modality imaging of extraskeletal Ewing sarcoma of the stomach","volume":"34","author":"Hopp","year":"2019","journal-title":"J Gastroenterol Hepatol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"666","DOI":"10.1097\/RLU.0000000000002659","article-title":"FDG PET\/CT in a case of primary pulmonary Ewing sarcoma","volume":"44","author":"Zhang","year":"2019","journal-title":"Clin Nucl Med"},{"key":"B12","doi-asserted-by":"publisher","first-page":"1343","DOI":"10.3892\/etm.2018.6323","article-title":"Personalized genomic analysis based on circulating tumor cells of extra-skeletal Ewing sarcoma of the uterus: a case report of a 16-year-old Korean female","volume":"16","author":"Lee","year":"2018","journal-title":"Exp Ther Med"},{"key":"B13","doi-asserted-by":"publisher","first-page":"465","DOI":"10.1007\/s00404-008-0898-7","article-title":"Extraskeletal neuroectodermal tumour of the vagina: a single case report and review","volume":"280","author":"Al-Tamimi","year":"2009","journal-title":"Arch Gynecol Obstet"},{"key":"B14","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1007\/s13193-011-0112-4","article-title":"Primary Ewing's sarcoma of penis - a rare case report","volume":"2","author":"Sharma","year":"2011","journal-title":"Indian J Surg Oncol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1007\/s11255-007-9202-1","article-title":"Ewing's sarcoma\/primitive neuroectodermal tumor (ES\/PNET) of the penis","volume":"40","author":"Kilicaslan","year":"2008","journal-title":"Int Urol Nephrol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1007\/s12262-015-1399-0","article-title":"Extraosseous Ewing sarcoma: diagnosis, prognosis and optimal management","volume":"78","author":"Galyfos","year":"2016","journal-title":"Indian J Surg"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1763","DOI":"10.1002\/cncr.28657","article-title":"Sarcoma classification: an update based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone","volume":"120","author":"Doyle","year":"2014","journal-title":"Cancer"},{"key":"B18","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1038\/modpathol.3800569","article-title":"Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma","volume":"19","author":"Olsen","year":"2006","journal-title":"Mod Pathol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"S47","DOI":"10.1038\/modpathol.2013.177","article-title":"Novel uses of immunohistochemistry in the diagnosis and classification of soft tissue tumors","volume":"27","author":"Hornick","year":"2014","journal-title":"Mod Pathol"},{"key":"B20","doi-asserted-by":"publisher","first-page":"294","DOI":"10.1056\/NEJM199408043310503","article-title":"The Ewing family of tumors\u2013a subgroup of small-round-cell tumors defined by specific chimeric transcripts","volume":"331","author":"Delattre","year":"1994","journal-title":"N Engl J Med"},{"key":"B21","doi-asserted-by":"publisher","first-page":"1771","DOI":"10.1002\/pbc.26096","article-title":"Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: a report from the Children's Oncology Group","volume":"63","author":"Cash","year":"2016","journal-title":"Pediatr Blood Cancer"},{"key":"B22","doi-asserted-by":"publisher","first-page":"3027","DOI":"10.1002\/cncr.25840","article-title":"Clinical features and outcomes in patients with extraskeletal Ewing sarcoma","volume":"117","author":"Applebaum","year":"2011","journal-title":"Cancer"},{"key":"B23","doi-asserted-by":"publisher","first-page":"783","DOI":"10.3348\/kjr.2015.16.4.783","article-title":"Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: a 17-year experience at a single institution","volume":"16","author":"Huh","year":"2015","journal-title":"Korean J Radiol"},{"key":"B24","doi-asserted-by":"publisher","first-page":"20140123","DOI":"10.1259\/bjr.20140123","article-title":"Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients","volume":"87","author":"Somarouthu","year":"2014","journal-title":"Br J Radiol"},{"key":"B25","doi-asserted-by":"publisher","first-page":"W1015","DOI":"10.2214\/AJR.11.6667","article-title":"A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses","volume":"197","author":"Javery","year":"2011","journal-title":"AJR Am J Roentgenol"},{"key":"B26","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-4419-0808-7","volume-title":"Bone Tumors: A Practical Guide to Imaging","author":"Wu","year":"2012"},{"key":"B27","doi-asserted-by":"publisher","first-page":"6227","DOI":"10.2147\/CMAR.S178979","article-title":"Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases","volume":"10","author":"Jiang","year":"2018","journal-title":"Cancer Manag Res"},{"key":"B28","doi-asserted-by":"publisher","first-page":"878","DOI":"10.1007\/s12094-019-02202-y","article-title":"Outcomes of extraskeletal vs. skeletal Ewing sarcoma patients treated with standard chemotherapy protocol","volume":"22","author":"Salah","year":"2020","journal-title":"Clin Transl Oncol"},{"key":"B29","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1016\/j.clon.2010.02.010","article-title":"Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institution","volume":"22","author":"El Weshi","year":"2010","journal-title":"Clin Oncol."},{"key":"B30","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1002\/pbc.24053","article-title":"Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement","volume":"59","author":"Applebaum","year":"2012","journal-title":"Pediatr Blood Cancer"},{"key":"B31","unstructured":"(NCCN) Clinical Practice Guidelines in Oncology Ewing sarcoma (Version 1. 2020)"},{"key":"B32","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1007\/s10147-012-0480-y","article-title":"Efficacy of carbon-ion radiotherapy and high-dose chemotherapy for patients with unresectable Ewing's sarcoma family of tumors","volume":"18","author":"Iwata","year":"2013","journal-title":"Int J Clin Oncol"},{"key":"B33","doi-asserted-by":"publisher","first-page":"1314","DOI":"10.1016\/j.ejca.2012.12.005","article-title":"Local control and sequelae in localised Ewing tumours of the spine: a French retrospective study","volume":"49","author":"Vogin","year":"2013","journal-title":"Eur J Cancer."}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2020.591257\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,7]],"date-time":"2021-01-07T06:09:41Z","timestamp":1609999781000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2020.591257\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,7]]},"references-count":33,"alternative-id":["10.3389\/fped.2020.591257"],"URL":"https:\/\/doi.org\/10.3389\/fped.2020.591257","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,7]]},"article-number":"591257"}}