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

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 loc...

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Detalles Bibliográficos
Autores principales: Palmerini, Emanuela, 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, Donati, Davide Maria, Ferrari, Stefano
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331738/
https://www.ncbi.nlm.nih.gov/pubmed/32616718
http://dx.doi.org/10.1038/s41598-020-66579-5
Descripción
Sumario: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.