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      Systemic therapy in primary angiosarcoma of the spleen

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          Abstract

          Primary splenic angiosarcoma is a very rare neoplasm with a high propensity for metastatic disease and poor prognosis. There is a paucity of literature concerning this specific sarcoma subtype and the role of systemic therapy is not well defined. A retrospective review of the prospectively maintained University of Washington/Seattle Cancer Care Alliance Sarcoma Unit database was performed to identify patients with splenic angiosarcoma treated between 2007 and 2012. In total there were 19 patients with angiosarcoma treated at the Seattle Cancer Care Alliance from 2007 to 2012. The number of patients with splenic angiosarcoma was 2 (11%). The first patient was a woman aged 57 years who was referred with metastatic splenic angiosarcoma to the liver, post-splenectomy. She was treated with 4 cycles of weekly paclitaxel prior to metastatic resection and 4 cycles of the same drug in an adjuvant scenario, achieving a pathological complete response to treatment. She is alive and on third-line systemic therapy. The second patient was a male patient aged 30 years who presented with metastatic high-grade splenic angiosarcoma and was treated with 3 lines of systemic therapy, including doxorubicin, paclitaxel and gemcitabine+docetaxel, but developed a gastrointestinal metastasis with subsequent gastrointestinal bleeding. Splenic angiosarcoma is a very rare neoplasm. Surgery remains the mainstay of management for localized disease. Paclitaxel administered weekly proved to be well-tolerated and resulted in a good radiological response in one of our patients, enabling resection of metastatic disease. Durable clinical benefit can be achieved in metastatic splenic angiosarcoma with multi modality management.

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          Most cited references 29

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          Angiosarcoma.

          Angiosarcomas are rare soft-tissue sarcomas of endothelial cell origin that have a poor prognosis. They can arise anywhere in the body, most commonly presenting as cutaneous disease in elderly white men, involving the head and neck and particularly the scalp. They can be caused by therapeutic radiation or chronic lymphoedema and hence secondary breast angiosarcomas are an important subgroup. Recent work has sought to establish the molecular biology of angiosarcomas and identify specific targets for treatment. Interest is now focused on trials of vascular-targeted drugs, which are showing promise in the control of angiosarcomas. In this review we discuss angiosarcoma and its current management, with a focus on clinical trials investigating the treatment of advanced disease. Copyright © 2010 Elsevier Ltd. All rights reserved.
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            Phase II study of sorafenib in patients with metastatic or recurrent sarcomas.

            PURPOSE Since activity of sorafenib was observed in sarcoma patients in a phase I study, we performed a multicenter phase II study of daily oral sorafenib in patients with recurrent or metastatic sarcoma. PATIENTS AND METHODS We employed a multiarm study design, each representing a sarcoma subtype with its own Simon optimal two-stage design. In each arm, 12 patients who received 0 to 1 prior lines of therapy were treated (0 to 3 for angiosarcoma and malignant peripheral-nerve sheath tumor). If at least one Response Evaluation Criteria in Solid Tumors (RECIST) was observed, 25 further patients with that sarcoma subtype were accrued. Results Between October 2005 and November 2007, 145 patients were treated; 144 were eligible for toxicity and 122 for response. Median age was 55 years; female-male ratio was 1.8:1. The median number of cycles was 3. Five of 37 patients with angiosarcoma had a partial response (response rate, 14%). This was the only arm to meet the RECIST response rate primary end point. Median progression-free survival was 3.2 months; median overall survival was 14.3 months. Adverse events (typically dermatological) necessitated dose reduction for 61% of patients. Statistical modeling in this limited patient cohort indicated sorafenib toxicity was correlated inversely to patient height. There was no correlation between phosphorylated extracellular signal regulated kinase expression and response in six patients with angiosarcoma with paired pre- and post-therapy biopsies. CONCLUSION As a single agent, sorafenib has activity against angiosarcoma and minimal activity against other sarcomas. Further evaluation of sorafenib in these and possibly other sarcoma subtypes appears warranted, presumably in combination with cytotoxic or kinase-specific agents.
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              Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.

              The objective of this phase II trial was to assess the efficacy and toxicity of weekly paclitaxel for patients with metastatic or unresectable angiosarcoma. Thirty patients were entered onto the study from April 2005 through October 2006. Paclitaxel was administered intravenously as a 60-minute infusion at a dose of 80 mg/m(2) on days 1, 8, and 15 of a 4-week cycle. The primary end point was the nonprogression rate after two cycles. The progression-free survival rates after 2 and 4 months were 74% and 45%, respectively. With a median follow-up of 8 months, the median time to progression was 4 months and the median overall survival was 8 months. The progression-free survival rate was similar in patients pretreated with chemotherapy and in chemotherapy-naïve patients (77% v 71%). Three patients with locally advanced breast angiosarcoma presented partial response, which enabled a secondary curative-intent surgery with complete histologic response in two cases. One toxic death occurred as a result of a thrombocytopenia episode. Six patients presented with grade 3 toxicities and one patient presented with a grade 4 toxicity. Anemia and fatigue were the most frequently reported toxicities. Weekly paclitaxel at the dose schedule used in the current study was well tolerated and demonstrated clinical benefit.
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                Author and article information

                Journal
                Rare Tumors
                RT
                RT
                Rare Tumors
                PAGEPress Publications (Pavia, Italy )
                2036-3605
                2036-3613
                13 November 2012
                10 October 2012
                : 4
                : 4
                Affiliations
                [1 ]School of Medicine, Federal University of Rio Grande do Sul, RS, Brazil;
                [2 ]Department of Hematology and Oncology, Sarcoma Unit, University of Washington, WA, USA;
                [3 ]Fred Hutchinson Cancer Research Center, Seattle, WA, USA;
                [4 ]Group Health Research Institute, Seattle, WA, USA
                Author notes
                Correspondence: Robin L. Jones, Seattle Cancer Care Alliance, 825 Eastlake Ave. E. Mailstop: G3630, 98109-1023 Seattle, USA. Tel. +1.206.2887439. E-mail: rjones@ 123456seattlecca.org

                Conflict of interests: the authors report no conflict of interests.

                Contributions: BPF and RLJ designed and wrote the manuscript; ETR, ETL and SMP treated the patients and reviewed the manuscript.

                Article
                rt.2012.e55
                10.4081/rt.2012.e55
                3557569
                23372919
                ©Copyright B. Pellini Ferreira et al., 2012

                This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0).

                Licensee PAGEPress, Italy

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                Categories
                Article

                Oncology & Radiotherapy

                splenic angiosarcoma, paclitaxel, anthracycline., systemic therapy

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