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      Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute

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          Abstract

          Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. The purpose of the present study was to report a large series of angiosarcoma at a single institution. Clinical data from 200 cases of angiosarcoma from the Shanghai Cancer Center (Shanghai, China) between March 2006 and March 2014 were retrospectively analyzed. The study population included 97 males and 103 females with ages between 4 and 91 years (median, 53 years). According to the tumor location, 200 cases were divided into 4 groups: i) Tumors involving the head and neck; ii) breast; iii) viscera (including internal organs and bone); and iv) soft tissue (including trunk and extremities). Of the 113 patients with follow-up data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5-years was 19.3% and the overall survival (OS) rate at 5-years was 40.8%. Site of tumor origin, size (≥5 cm) and histological differentiation influenced DFS (P=0.032, 0.038 and <0.001, respectively), and OS (P<0.001, 0.008 and <0.001, respectively) rates. Age (<65 years) and multimodal treatment correlated with improved OS (P=0.003 and <0.001, respectively). Tumor differentiation and treatment modality were identified to be independent determinants of OS (P<0.001 and 0.038, respectively). Tumor recurrence/metastasis was an independent predictor of DFS (P<0.001). The prognosis of angiosarcoma is poor and the mortality rate is high. The site of tumor origin, size, histological differentiation, age, treatment modality and tumor recurrence/metastasis are all significant prognostic factors. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.

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          Most cited references31

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          Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases.

          Angiosarcomas are rare, heterogeneous and a retrospective study was conducted to describe their natural history. We reviewed 161 files of angiosarcoma treated in three institutions of the French Sarcoma Group from 1980 to 2004. Survival and prognostic factors for survival were analyzed. Median age was 52 years. Primary sites were the breast (35%), skin (20%) and soft tissues (13%). At initial diagnosis, 31 (19%) had metastases. Surgery was the first treatment in 121 (75%) patients combined with chemotherapy or radiotherapy in 34 and 32, respectively. Ninety (74%) of these 121 patients relapsed, mostly locally (50). With an average time since initial diagnosis of 8.1 years, 123 (76%) patients progressed and 76 (47%) died. Median survival was 3.4 years [95% confidence interval (CI) 2.4-5.8], and the 5-year overall survival (OS) rate was 43% (95% CI 33-53). In multivariate analysis, liver primary site [relative risk (RR) = 12.62], performance status (PS) of two or more (RR = 3.83), presence of metastases at diagnosis (RR = 2.50), soft tissue tumor (RR = 0.31) were correlated to OS. PS, liver and soft tissue tumors were identified as independent prognostic factors for progression-free survival. Angiosarcomas have an overall poor outcome, but with a clearly distinct prognosis depending on the primary site.
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            Angiosarcoma. A report of 67 patients and a review of the literature.

            Angiosarcomas (AS) are rare, aggressive tumors. Optimal treatment has not been well defined. The authors undertook a retrospective review of patients seen at their institution with the intent of identifying prognostic factors and optimal treatment strategies. Between 1955 and 1990, 67 patients with AS were seen at the University of California, at Los Angeles Medical Center. Follow-up ranged from 1 to 173 months with a median of 30 months. The overall prognosis was poor. The actuarial 2- and 5-year disease free survivals (DFS) were 44% and 24%, respectively. Of 52 recurrences after primary treatment, 81% (42 of 52) had a component of local failure. Twenty-eight patients had developed distant metastases at last follow-up. Of patients who received surgery (S) and radiation therapy (RT), with or without chemotherapy (CT), 5-year actuarial DFS was 43%, compared with 17% for patients who underwent S +/- CT as initial treatment (P = 0.03). Only 9% of patients (1 of 11) treated with RT +/- CT were rendered free of disease. Patients with AS usually present with high grade histology, and with multifocal disease. There is a propensity for both local recurrence and distant metastases. Our results and a review of the literature, suggest that S plus RT offers the best chance for long term control of this aggressive tumor. The role of CT remains undefined.
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              A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy.

              Angiosarcoma is a rare vascular malignancy, and there are few published data to guide chemotherapy treatment decisions. We present a retrospective analysis of angiosarcoma encompassing all anatomic sites of disease presenting to a single institution over a 14-year period. Characteristics at presentation and prognostic factors are reviewed. For patients with unresectable disease, progression-free survival with various chemotherapy regimens is described. Pathological confirmation of all cases was performed before they were included in this analysis. One hundred twenty-five patients with angiosarcoma were seen and treated between January 1, 1990 and December 31, 2003. Angiosarcoma showed marked variation by anatomic site regarding gender ratio, median age at diagnosis, overall survival, and response to chemotherapy. Overall 5-year survival was 31% for angiosarcoma. Superficial depth and negative microscopic surgical margins correlated with longer overall survival, but tumor size did not reach significance as a prognostic factor. For unresectable angiosarcoma, doxorubicin based regimens yielded progression-free survival of 3.7-5.4 months. Paclitaxel achieved a progression-free survival of 6.8 months for scalp angiosarcoma and 2.8 months for sites below the clavicle. Angiosarcoma is an aggressive malignancy characterized by biologic heterogeneity at different anatomic sites and relative sensitivity to paclitaxel and doxorubicin.
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                Author and article information

                Journal
                Oncol Lett
                Oncol Lett
                OL
                Oncology Letters
                D.A. Spandidos
                1792-1074
                1792-1082
                November 2017
                06 September 2017
                06 September 2017
                : 14
                : 5
                : 5370-5378
                Affiliations
                [1 ]Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, P.R. China
                [2 ]Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200433, P.R. China
                Author notes
                Correspondence to: Dr Jian Wang, Department of Pathology, Fudan University Shanghai Cancer Center, 270 Dong An Street, Shanghai 200032, P.R. China, E-mail: softtissuetumor@ 123456163.com
                [*]

                Contributed equally

                Article
                OL-0-0-6892
                10.3892/ol.2017.6892
                5656021
                29113171
                5dbd9e66-b173-4989-afb3-032ba301cc56
                Copyright: © Wang et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

                History
                : 18 December 2015
                : 23 May 2017
                Categories
                Articles

                Oncology & Radiotherapy
                angiosarcoma,soft tissue sarcoma,clinicopathological features,prognostic factors,retrospective analysis

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