Angiosarcoma of bone: a retrospective study of the European Musculoskeletal Oncology Society (EMSOS)

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Palmerini , E , Leithner , A , Windhager , R , Gosheger , G , Boye , K , Laitinen , M , Hardes , J , Traub , F , Jutte , P , Willegger , M , Casanova , J , Setola , E , Righi , A , Picci , P , Donatih , D M & Ferrari , S 2020 , ' Angiosarcoma of bone: a retrospective study of the European Musculoskeletal Oncology Society (EMSOS) ' , Scientific Reports , vol. 10 , no. 1 , pp. 10853 .

Title: Angiosarcoma of bone: a retrospective study of the European Musculoskeletal Oncology Society (EMSOS)
Author: Palmerini, Emmanuela; Leithner, Andreas; Windhager, Reinhard; Gosheger, Georg; Boye, Kjetil; Laitinen, Minna; Hardes, Jendrik; Traub, Frank; Jutte, Paul; Willegger, Madeleine; Casanova, Jose; Setola, Elisabetta; Righi, Alberto; Picci, Piero; Donatih, Davide Maria; Ferrari, Stefano
Contributor organization: HUS Musculoskeletal and Plastic Surgery
I kirurgian klinikka (Töölö)
Department of Surgery
Helsinki University Hospital Area
University of Helsinki
Date: 2020-07-02
Language: eng
Number of pages: 8
Belongs to series: Scientific Reports
ISSN: 2045-2322
Abstract: Angiosarcoma of bone (B-AS) is a rare malignant tumor of vascular origin. The aim of this retrospective study is to report on treatments and prognosis. Data were collected from the EMSOS website. 80 patients in 9 centers included: 51 male/29 female; median age 54 years (range 17 to 92); 56% with localized disease, 44% metastatic. Primary tumor surgery: 76% (30% amputation, 26% intralesional margins); radiotherapy (RT): 41%; chemotherapy (CT): 47% (56% in metastatic, 41% in localized cases). With a median follow-up of 31 months (range 40 to 309), 5-year overall survival (OS) was 27% (95%CI 16-30): 41% (95%CI 25-56) for localized patients, and 8% (95%CI 0-20) for metastatic (p = 0.002). In metastatic patients, 1 year OS was significantly influenced by chemotherapy response: 67% (95CI% 29-100) for those who responded or had stable disease (n = 7), and 18% (95CI% 0-41) for patients with progressive disease (n = 11), p 0.002. The surgical complete remission (SCR) status was pivotal in localized patients (5-year OS 45% for SCR, 17% no SCR, p = 0.03); also 5-year OS was significantly influenced by age and site of the tumor. After multivariate analysis, the addition of radiotherapy to surgery significantly influenced the disease-free survival (DFS) rate, whereas the use of chemotherapy lost the significance showed at the univariate analysis. Overall, patients with metastatic B-AS have a dismal prognosis, with a prolonged survival in case with a response to chemotherapy. Experimental trials with more active systemic treatment regimens are needed. In patients with localized disease, the patient's age and site of the tumor are prognostic factors and any effort must be made to achieve an SCR status. No definitive conclusions can be drawn from our data on the use of adjuvant chemotherapy, while the use of adjuvant radiotherapy might improve DSF in patients surgically free of disease.
Subject: 3122 Cancers
3126 Surgery, anesthesiology, intensive care, radiology
Peer reviewed: Yes
Rights: cc_by
Usage restriction: openAccess
Self-archived version: publishedVersion

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