{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T18:08:31Z","timestamp":1792692511879,"version":"4.5.0"},"reference-count":22,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2023,4,22]],"date-time":"2023-04-22T00:00:00Z","timestamp":1682121600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,6,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Given the rarity of cutaneous\/subcutaneous Ewing sarcoma, their clinical characteristics remain poorly understood. In this study, we aimed to analyse the clinical characteristics of patients with cutaneous\/subcutaneous Ewing sarcoma and review the treatment strategy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed the clinical data of 154 patients with Ewing sarcoma who were treated at our hospital between 2005 and 2019. Amongst these patients, 21 patients with cutaneous\/subcutaneous Ewing sarcoma were analysed. As a basic strategy, patients with localized disease received intensive chemotherapy (vincristine\u2013doxorubicin\u2013cyclophosphamide\/ifosfamide\u2013etoposide), followed by definitive surgery with or without radiotherapy. In total, 15 patients underwent pre-diagnostic resection with macroscopic residue (seven patients) or non-macroscopic residue (eight patients) before intensive chemotherapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The median tumour length of the measurable lesions was 3.2\u00a0cm, and the ratio of metastasis was significantly lower than the Ewing sarcoma of other anatomical sites (10% vs. 37%, P\u00a0=\u00a00.013). Despite the pre-diagnostic resection, local recurrence after additional resection and\/or adjuvant radiotherapy did not occur in any of the patients with localized disease. Overall survival was significantly higher in patients with cutaneous\/subcutaneous Ewing sarcoma than in patients with Ewing sarcoma of other anatomical sites (hazard ratio\u00a0=\u00a00.33, P\u00a0=\u00a00.013). The event-free survival rate of cutaneous\/subcutaneous Ewing sarcoma was also superior to that of Ewing sarcoma of other anatomical sites (hazard ratio\u00a0=\u00a00.35, P\u00a0=\u00a00.01).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Patients with cutaneous\/subcutaneous Ewing sarcoma may have better prognosis than those with Ewing sarcoma at other anatomical sites. Although pre-diagnostic resection without appropriate investigations is not recommended, local control may be recovered by using a combination of additional resection, chemotherapy and radiotherapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/jjco\/hyad031","type":"journal-article","created":{"date-parts":[[2023,3,27]],"date-time":"2023-03-27T13:21:00Z","timestamp":1679923260000},"page":"604-610","source":"Crossref","is-referenced-by-count":7,"title":["Clinical characteristics of primary cutaneous and subcutaneous Ewing sarcoma"],"prefix":"10.1093","volume":"53","author":[{"given":"Hisaki","family":"Aiba","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"},{"name":"Department of Orthopaedic Surgery, Nagoya City University , Nagoya , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuki","family":"Kojima","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsunori","family":"Shimoi","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuki","family":"Sudo","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shu","family":"Yazaki","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Imai","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiko","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shintaro","family":"Iwata","sequence":"additional","affiliation":[{"name":"Department of Musculoskeletal Oncology and Rehabilitation, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eisuke","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Musculoskeletal Oncology and Rehabilitation, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Kawai","sequence":"additional","affiliation":[{"name":"Department of Musculoskeletal Oncology and Rehabilitation, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayumu","family":"Arakawa","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chitose","family":"Ogawa","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Kimura","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery, Nagoya City University , Nagoya , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kan","family":"Yonemori","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, National Cancer Center Hospital , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,4,22]]},"reference":[{"key":"2023063007014868800_ref1","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1038\/s41572-018-0003-x","volume":"4","author":"Gr\u00fcnewald","year":"2018","journal-title":"Ewing sarcoma Nat Rev Dis Primers"},{"key":"2023063007014868800_ref2","doi-asserted-by":"crossref","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":"2023063007014868800_ref3","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/S0360-3016(99)00391-0","article-title":"Cutaneous and subcutaneous Ewing's sarcoma: an indolent disease","volume":"46","author":"Chow","year":"2000","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2023063007014868800_ref4","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1111\/j.1365-2133.2011.10743.x","article-title":"Primary cutaneous Ewing sarcoma: a systematic review focused on treatment and outcome","volume":"166","author":"Delaplace","year":"2012","journal-title":"Br J Dermatol"},{"key":"2023063007014868800_ref5","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1002\/pbc.25535","article-title":"Primary cutaneous and subcutaneous Ewing sarcoma","volume":"62","author":"Di Giannatale","year":"2015","journal-title":"Pediatr Blood Cancer"},{"key":"2023063007014868800_ref6","doi-asserted-by":"crossref","first-page":"4148","DOI":"10.1200\/JCO.2011.41.5703","article-title":"Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's oncology group","volume":"30","author":"Womer","year":"2012","journal-title":"J Clin Oncol"},{"key":"2023063007014868800_ref7","doi-asserted-by":"crossref","first-page":"2511","DOI":"10.1002\/cncr.10492","article-title":"TNM residual tumor classification revisited","volume":"94","author":"Wittekind","year":"2002","journal-title":"Cancer"},{"key":"2023063007014868800_ref8","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1093\/jjco\/hyn153","article-title":"Preoperative and postoperative chemotherapy with ifosfamide and adriamycin for adult high-grade soft-tissue sarcomas in the extremities: Japan clinical oncology group study JCOG0304","volume":"39","author":"Tanaka","year":"2009","journal-title":"Jpn J Clin Oncol"},{"key":"2023063007014868800_ref9","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.ejca.2008.10.026","article-title":"New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1)","volume":"45","author":"Eisenhauer","year":"2009","journal-title":"Eur J Cancer"},{"key":"2023063007014868800_ref10","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.ijrobp.2015.01.022","article-title":"Prognostic factors and patterns of relapse in Ewing sarcoma patients treated with chemotherapy and r0 resection","volume":"92","author":"Pan","year":"2015","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2023063007014868800_ref11","doi-asserted-by":"crossref","first-page":"185","DOI":"10.2106\/00004623-196143020-00004","article-title":"A critical analysis of 165 cases","volume":"43\u2013A","author":"Dahlin","year":"1961","journal-title":"J Bone Joint Surg Am"},{"key":"2023063007014868800_ref12","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1200\/JCO.1997.15.2.574","article-title":"Ewing's sarcoma of soft tissues in childhood: a report from the intergroup rhabdomyosarcoma study, 1972 to 1991","volume":"15","author":"Raney","year":"1997","journal-title":"J Clin Oncol"},{"key":"2023063007014868800_ref13","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1038\/ng.1107","article-title":"A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion","volume":"44","author":"Pierron","year":"2012","journal-title":"Nat Genet"},{"key":"2023063007014868800_ref14","doi-asserted-by":"crossref","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":"2023063007014868800_ref15","first-page":"319","article-title":"Five-year survival of patients with Ewing's sarcoma","volume":"124","author":"Falk","year":"1967","journal-title":"Surg Gynecol Obstet"},{"key":"2023063007014868800_ref16","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1016\/j.jos.2020.12.025","article-title":"The incidence of unplanned excision in patients with soft tissue sarcoma: reports from the bone and soft tissue tumor registry in Japan","volume":"27","author":"Nakamura","year":"2022","journal-title":"J Orthop Sci"},{"key":"2023063007014868800_ref17","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1002\/cncr.29065","article-title":"Comparative evaluation of local control strategies in localized Ewing sarcoma of bone: a report from the Children's oncology group","volume":"121","author":"DuBois","year":"2015","journal-title":"Cancer"},{"key":"2023063007014868800_ref18","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1016\/j.humpath.2011.04.023","article-title":"The CIC-DUX4 fusion transcript is present in a subgroup of pediatric primitive round cell sarcomas","volume":"43","author":"Graham","year":"2012","journal-title":"Hum Pathol"},{"key":"2023063007014868800_ref19","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1111\/cup.13656","article-title":"Superficial sarcomas with CIC rearrangement are aggressive neoplasms: a series of eight cases","volume":"47","author":"Ko","year":"2020","journal-title":"J Cutan Pathol"},{"key":"2023063007014868800_ref20","doi-asserted-by":"crossref","DOI":"10.1101\/mcs.a005942","article-title":"Clinicopathologic and molecular analysis of a BCOR-CCNB3 undifferentiated sarcoma of the kidney reveals significant epigenetic alterations","volume":"8","author":"Hagoel","year":"2022","journal-title":"Cold Spring Harb Mol Case Stud"},{"key":"2023063007014868800_ref21","doi-asserted-by":"crossref","first-page":"7999","DOI":"10.1002\/jcp.27558","article-title":"Ewing-like sarcoma: an emerging family of round cell sarcomas","volume":"234","author":"Renzi","year":"2019","journal-title":"J Cell Physiol"},{"key":"2023063007014868800_ref22","doi-asserted-by":"crossref","first-page":"2191","DOI":"10.1002\/pbc.25210","article-title":"Ewing-like sarcomas with BCOR-CCNB3 fusion transcript: a clinical, radiological and pathological retrospective study from the Soci\u00e9t\u00e9 Fran\u00e7aise des cancers de L'Enfant","volume":"61","author":"Cohen-Gogo","year":"2014","journal-title":"Pediatr Blood Cancer"}],"container-title":["Japanese Journal of Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jjco\/article-pdf\/53\/7\/604\/50738051\/hyad031.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jjco\/article-pdf\/53\/7\/604\/50738051\/hyad031.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,6,30]],"date-time":"2023-06-30T03:02:34Z","timestamp":1688094154000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jjco\/article\/53\/7\/604\/7136607"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,22]]},"references-count":22,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2023,4,22]]},"published-print":{"date-parts":[[2023,6,29]]}},"URL":"https:\/\/doi.org\/10.1093\/jjco\/hyad031","relation":{},"ISSN":["1465-3621"],"issn-type":[{"value":"1465-3621","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,7]]},"published":{"date-parts":[[2023,4,22]]}}}