{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:15:37Z","timestamp":1792034137295,"version":"4.4.0"},"reference-count":37,"publisher":"Wiley","license":[{"start":{"date-parts":[[2021,9,10]],"date-time":"2021-09-10T00:00:00Z","timestamp":1631232000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Sarcoma"],"published-print":{"date-parts":[[2021,9,10]]},"abstract":"<jats:p>Background. Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. Methods. We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan\u2013Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. Results. The median age was 67 years (19\u201372.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size &lt;10\u2009cm (<jats:inline-formula>\n                     <a:math xmlns:a=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\">\n                        <a:mi>p<\/a:mi>\n                     <\/a:math>\n                  <\/jats:inline-formula>\u2009=\u20090.009), negative surgical margins (<jats:inline-formula>\n                     <c:math xmlns:c=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M2\">\n                        <c:mi>p<\/c:mi>\n                     <\/c:math>\n                  <\/jats:inline-formula>\u2009=\u20090.021), and negative lymph node status (<jats:inline-formula>\n                     <e:math xmlns:e=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M3\">\n                        <e:mi>p<\/e:mi>\n                     <\/e:math>\n                  <\/jats:inline-formula>\u2009=\u20090.007). LRFS and MFS were longer for tumor size &lt;10\u2009cm (<jats:inline-formula>\n                     <g:math xmlns:g=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M4\">\n                        <g:mi>p<\/g:mi>\n                     <\/g:math>\n                  <\/jats:inline-formula>\u2009=\u20090.012 and <jats:inline-formula>\n                     <i:math xmlns:i=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M5\">\n                        <i:mi>p<\/i:mi>\n                     <\/i:math>\n                  <\/jats:inline-formula>\u2009=\u20090.013). In multivariate analyses, age &lt;70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; <jats:inline-formula>\n                     <k:math xmlns:k=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M6\">\n                        <k:mi>p<\/k:mi>\n                     <\/k:math>\n                  <\/jats:inline-formula>\u2009=\u20090.035). Conclusions. Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head\/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.<\/jats:p>","DOI":"10.1155\/2021\/9960085","type":"journal-article","created":{"date-parts":[[2021,9,11]],"date-time":"2021-09-11T19:35:14Z","timestamp":1631388914000},"page":"1-10","source":"Crossref","is-referenced-by-count":12,"title":["Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology"],"prefix":"10.1155","volume":"2021","author":[{"given":"Inna","family":"Schott","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sven-Thorsten","family":"Liffers","sequence":"additional","affiliation":[{"name":"Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"Division of Solid Tumor Translational Oncology, German Cancer Consortium (DKTK,Partner Site Essen) and German Cancer Research Center, DKFZ, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4727-5699","authenticated-orcid":true,"given":"Farhad","family":"Farzaliyev","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4738-7121","authenticated-orcid":true,"given":"Johanna","family":"Falkenhorst","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans-Ulrich","family":"Steinau","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantion Surgery, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9817-9365","authenticated-orcid":true,"given":"J\u00fcrgen-Walter","family":"Treckmann","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantion Surgery, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1011-7822","authenticated-orcid":true,"given":"Lars Erik","family":"Podleska","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Oncology, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9224-9318","authenticated-orcid":true,"given":"Christoph","family":"P\u00f6ttgen","sequence":"additional","affiliation":[{"name":"Department of Radiotherapy, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9856-7050","authenticated-orcid":true,"given":"Hans-Ulrich","family":"Schildhaus","sequence":"additional","affiliation":[{"name":"Institute of Pathology, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4313-0895","authenticated-orcid":true,"given":"Marit","family":"Ahrens","sequence":"additional","affiliation":[{"name":"Medical Clinic II, University Hospital Frankfurt, Frankfurt am Main, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5435-7860","authenticated-orcid":true,"given":"Uta","family":"Dirksen","sequence":"additional","affiliation":[{"name":"Pediatrics III Pediatric Hematology, Oncology, Immunology, Cardiology, Pulmonology, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2480-1166","authenticated-orcid":true,"given":"Fatma-Zehra","family":"Murat","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8772-4778","authenticated-orcid":true,"given":"Jens T.","family":"Siveke","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"Bridge Institute of Experimental Tumor Therapy, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"Division of Solid Tumor Translational Oncology, German Cancer Consortium (DKTK,Partner Site Essen) and German Cancer Research Center, DKFZ, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5949-8120","authenticated-orcid":true,"given":"Sebastian","family":"Bauer","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9655-1226","authenticated-orcid":true,"given":"Rainer","family":"Hamacher","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Sarcoma Center, West German Cancer Center, University Hospital Essen, Essen, Germany"},{"name":"German Cancer Consortium (DKTK), Partner Site University Hospital Essen, Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1016\/s1470-2045(10)70023-1"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0246958"},{"key":"3","article-title":"Soft tissue sarcoma incidence and survival tumours diagnosed in england between 1985 and 2009","author":"M. 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