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      2018 ESMO Sarcoma and GIST Symposium: ‘take-home messages’ in soft tissue sarcoma

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          Abstract

          The 7th edition of the ‘ESMO Sarcoma and GIST Symposium’ was held in Milan in February 2018. For the first time, the Symposium brought together representatives from the European Reference Network on rare adult solid cancer (EURACAN) joined by sarcoma experts from the USA, Japan and patient advocacy groups, to share insights and discuss future directions in this rare condition. This commentary will summarise the highlights in soft tissue sarcomas.

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          Targeting EZH2 in cancer.

          Kimberly H Kim, Charles Roberts (2016)
          Recent genomic studies have resulted in an emerging understanding of the role of chromatin regulators in the development of cancer. EZH2, a histone methyl transferase subunit of a Polycomb repressor complex, is recurrently mutated in several forms of cancer and is highly expressed in numerous others. Notably, both gain-of-function and loss-of-function mutations occur in cancers but are associated with distinct cancer types. Here we review the spectrum of EZH2-associated mutations, discuss the mechanisms underlying EZH2 function, and synthesize a unifying perspective that the promotion of cancer arises from disruption of the role of EZH2 as a master regulator of transcription. We further discuss EZH2 inhibitors that are now showing early signs of promise in clinical trials and also additional strategies to combat roles of EZH2 in cancer.
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            Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial.

            Ian Judson, Jaap Verweij, Hans Gelderblom … (2014)
            Effective targeted treatment is unavailable for most sarcomas and doxorubicin and ifosfamide-which have been used to treat soft-tissue sarcoma for more than 30 years-still have an important role. Whether doxorubicin alone or the combination of doxorubicin and ifosfamide should be used routinely is still controversial. We assessed whether dose intensification of doxorubicin with ifosfamide improves survival of patients with advanced soft-tissue sarcoma compared with doxorubicin alone.
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              Molecularly targeted therapy based on tumour molecular profiling versus conventional therapy for advanced cancer (SHIVA): a multicentre, open-label, proof-of-concept, randomised, controlled phase 2 trial.

              Christophe Le Tourneau, Jean-Pierre Delord, Anthony Goncalves … (2015)
              Molecularly targeted agents have been reported to have anti-tumour activity for patients whose tumours harbour the matching molecular alteration. These results have led to increased off-label use of molecularly targeted agents on the basis of identified molecular alterations. We assessed the efficacy of several molecularly targeted agents marketed in France, which were chosen on the basis of tumour molecular profiling but used outside their indications, in patients with advanced cancer for whom standard-of-care therapy had failed.
              Article 0 comments Cited 405 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): ESMO Open
                Journal ID (iso-abbrev): ESMO Open
                Journal ID (hwp): esmoopen
                Journal ID (publisher-id): esmoopen
                Title: ESMO Open
                Publisher: BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                ISSN (Electronic): 2059-7029
                Publication date Collection: 2018
                Publication date (Electronic): 28 June 2018
                Volume: 3
                Issue: 4
                Electronic Location Identifier: e000390
                Affiliations
                [1 ]departmentDepartment of Medical Oncology , IRCCS Fondazione Istituto Nazionale Tumori , Milano, Italy
                [2 ]departmentSarcoma Unit , Royal Marsden NHS Foundation Trust and Institute of Cancer Research , London, UK
                [3 ]departmentDepartment of Surgery A , Tel- Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel- Aviv University , Tel- Aviv, Israel
                [4 ]departmentDepartment of Pathology , Treviso Regional Hospital , Treviso, Italy
                [5 ]departmentSarcoma Surgery , IRCCS Fondazione Istituto Nazionale Tumori , Milan, Italy
                [6 ]departmentDepartment of Medical Oncology and Haemato-Oncology , University of Milan , Milan, Italy
                Author notes
                [Correspondence to ] Dr Anna Maria Frezza; annamaria.frezza@ 123456istitutotumori.mi.it
                Article
                Publisher ID: esmoopen-2018-000390
                DOI: 10.1136/esmoopen-2018-000390
                PMC ID: 6045770
                PubMed ID: 30018812
                SO-VID: df38424a-28d7-4e2a-a0b3-1d3824c5b0b5
                Copyright © © European Society for Medical Oncology (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
                License:

                This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

                History
                Date received : 27 April 2018
                Date revision received : 08 May 2018
                Date accepted : 11 May 2018
                Categories
                Subject: Review
                Subject: 1506
                Custom metadata
                special-feature unlocked

                Keywords: soft tissue sarcoma,euracan,epigenetics,immunotherapy,massive parallel sequencing
                Data availability:
                Keywords: soft tissue sarcoma, euracan, epigenetics, immunotherapy, massive parallel sequencing

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