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      Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?

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          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor with indolent course. Liver transplantation for local disease is the treatment of choice. In the metastatic setting there is no consensus regarding the appropriate systemic treatment. We present two cases of metastatic hepatic epithelioid hemangioendothelioma (hEHE) treated with the combination of Doxorubicin and Olaratumab. Both patients showed Stable Disease (SD) as a response, after the completion of six cycles of this combination therapy.

          Most cited references32

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          The Hippo pathway effectors TAZ and YAP in development, homeostasis and disease.

          Xaralabos Varelas (2014)
          Studies over the past 20 years have defined the Hippo signaling pathway as a major regulator of tissue growth and organ size. Diverse roles for the Hippo pathway have emerged, the majority of which in vertebrates are determined by the transcriptional regulators TAZ and YAP (TAZ/YAP). Key processes regulated by TAZ/YAP include the control of cell proliferation, apoptosis, movement and fate. Accurate control of the levels and localization of these factors is thus essential for early developmental events, as well as for tissue homeostasis, repair and regeneration. Recent studies have revealed that TAZ/YAP activity is regulated by mechanical and cytoskeletal cues as well as by various extracellular factors. Here, I provide an overview of these and other regulatory mechanisms and outline important developmental processes controlled by TAZ and YAP.
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            Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

            Margaret von Mehren, R. Randall, Robert Benjamin … (2018)
            Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.
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              An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas.

              M Agulnik, J.L. Yarber, S.H. Okuno … (2013)
              To determine efficacy and safety of bevacizumab, a recombinant humanized antibody against vascular endothelial growth factor (VEGF), in the treatment of metastatic or locally advanced angiosarcoma and epithelioid hemangioendotheliomas. In this single-arm phase II trial, 32 patients were enrolled and they received bevacizumab 15 mg/kg IV infusion in 21-day cycles. Patients had disease that was deemed not surgically resectable, Eastern Cooperative Oncology Group (ECOG) performance status of ≤1, adequate organ function and had not received any radiation treatment in the last 28 days. Of the 30 patients evaluated for efficacy and toxic effect, four (two angiosarcoma and two epithelioid hemangioendothelioma; 17%) had a partial response. Fifteen patients (11 angiosarcoma and 4 epithelioid hemangioendothelioma; 50%) showed stable disease with a mean time to progression of 26 weeks. Bevacizumab was well tolerated with only one grade 4 adverse event. Expected known toxic effects of the drug were manageable. Bevacizumab is an effective and well-tolerated treatment for metastatic or locally advanced angiosarcoma and epithelioid hemangioendotheliomas. Further phase III studies of bevacizumab in combination with other chemotherapeutic agents and/or radiation treatment are warranted.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Ther Clin Risk Manag
                Journal ID (iso-abbrev): Ther Clin Risk Manag
                Journal ID (publisher-id): TCRM
                Journal ID (pmc): tcriskman
                Title: Therapeutics and Clinical Risk Management
                Publisher: Dove
                ISSN (Print): 1176-6336
                ISSN (Electronic): 1178-203X
                Publication date (Electronic): 27 February 2020
                Publication date Collection: 2020
                Volume: 16
                Pages: 141-146
                Affiliations
                [1 ]Department of Clinical Therapeutics, General Hospital Alexandra , Athens, Greece
                [2 ]Department of Genetics, Agios Savvas Hospital , Athens, Greece
                [3 ]Department of Pathology Aretaion Hospital, National & Kapodistrian University of Athens , Athens, Greece
                [4 ]Institute of Cellular Medicine, Newcastle University , Newcastle upon Tyne, UK
                Author notes
                Correspondence: Anastasios Kyriazoglou Vasilisis Sofias 80, Athens11528, GreeceTel +302132162545Fax +302132162511 Email tassoskyr@gmail.com
                Author information
                http://orcid.org/0000-0001-7192-4575
                Article
                Publisher ID: 220804
                DOI: 10.2147/TCRM.S220804
                PMC ID: 7051805
                SO-VID: 32ea16b1-cca6-4d7c-9c46-a5a4b812e3ec
                Copyright © © 2020 Kyriazoglou et al.
                License:

                This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms ( https://www.dovepress.com/terms.php).

                History
                Date received : 25 June 2019
                Date accepted : 12 November 2019
                Page count
                Figures: 2, References: 43, Pages: 6
                Categories
                Subject: Case Series

                ScienceOpen disciplines: Medicine
                Keywords: olaratumab and doxorubicin,metastatic hepatic epithelioid hemangioendothelioma,1st line treatment
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: olaratumab and doxorubicin, metastatic hepatic epithelioid hemangioendothelioma, 1st line treatment

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