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<title>Lymphangiosarcoma</title>

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<span id="openzim-page-title" class="mw-page-title-main"><span class="mw-page-title-main">Lymphangiosarcoma</span></span>

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</style><table class="infobox infobox-has-images-with-white-backgrounds"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Lymphangiosarcoma</th></tr><tr><th scope="row" class="infobox-label"><a href="Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="Oncology" title="Oncology">Oncology</a> <span class="penicon autoconfirmed-show"></span></td></tr></tbody></table><style data-mw-deduplicate="TemplateStyles:r1236303919">

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<p><b>Lymphangiosarcoma</b> is a rare <a href="Cancer" title="Cancer">cancer</a> which occurs in long-standing cases of primary or secondary <a href="Lymphedema" title="Lymphedema">lymphedema</a> (swelling due to <a href="Lymphatic_system" title="Lymphatic system">lymphatic system</a> obstruction). It involves either the upper or lower lymphedematous extremities but is most common in upper extremities.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> Although its name implies lymphatic origin, it is believed to arise from <a href="Endothelial_cell" class="mw-redirect" title="Endothelial cell">endothelial cells</a> and may be more accurately referred to as <a href="Angiosarcoma" title="Angiosarcoma">angiosarcoma</a>.

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<div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2></div>

<p>Lymphangiosarcoma may present as a purple discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting to an extensive necrotic focus involving the skin and subcutaneous tissue.<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> It metastasizes quickly. Lymphangiosarcoma mostly occurs in the upper arm, forearm, elbow, and anterior chest wall. Furthermore, in post-mastectomy patients, lymphangiosarcoma is associated with sarcomatous degeneration, especially in patients who have survived five or more years and who have severe lymphedema.<sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Causes">Causes</h2></div>

<p>It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical <a href="Mastectomy" title="Mastectomy">Halstedian radical mastectomy</a>, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment—and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as <a href="Stewart%E2%80%93Treves_syndrome" title="Stewart–Treves syndrome">Stewart–Treves syndrome</a>. The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Schreiber <i>et al.</i> proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup>

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<div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2></div>

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<div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2></div>

<p>The most successful treatment for lymphangiosarcoma is amputation of the affected limb if possible. <a href="Chemotherapy" title="Chemotherapy">Chemotherapy</a> may be administered if there is evidence or suspicion of <a href="Metastasis" title="Metastasis">metastatic disease</a>. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as <a href="Paclitaxel" title="Paclitaxel">paclitaxel</a>,<sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> <a href="Doxorubicin" title="Doxorubicin">doxorubicin</a>,<sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> <a href="Ifosfamide" title="Ifosfamide">ifosfamide</a>, and <a href="Gemcitabine" title="Gemcitabine">gemcitabine</a><sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> exhibit antitumor activity.

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<ul><li><a href="Angiosarcoma" title="Angiosarcoma">Angiosarcoma</a></li>

<li><a href="Hemangiosarcoma" title="Hemangiosarcoma">Hemangiosarcoma</a></li>

<li><a href="Lymphangioma" class="mw-redirect" title="Lymphangioma">Lymphangioma</a></li>

<li><a href="Sarcoma" title="Sarcoma">Sarcoma</a></li>

<li><a href="Stewart%E2%80%93Treves_syndrome" title="Stewart–Treves syndrome">Stewart–Treves syndrome</a></li></ul>

<div class="mw-heading mw-heading2"><h2 id="References">References</h2></div>

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<li id="cite_note-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-1">^</a></b></span> <span class="reference-text">Hellman S, DeVita VT, Rosenberg S (2001). Cancer: principles & practice of oncology. Philadelphia: Lippincott-Raven. p. 1853. <style data-mw-deduplicate="TemplateStyles:r1238218222">

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</style><a href="ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <bdi>0-7817-2229-2</bdi>.</span>

</li>

<li id="cite_note-2"><span class="mw-cite-backlink"><b><a href="#cite_ref-2">^</a></b></span> <span class="reference-text"><cite class="citation book cs1"><a rel="nofollow" class="external text" href="https://web.archive.org/web/20140426232634/https://www.lymphedemastore.com/ViewProducts.aspx?cid=106"><i>LYMPHEDEMA MANAGEMENT</i></a>. Academy of Lymphatic Studies 2014. 2014. pp. <span class="nowrap">26–</span>30. <a href="ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <bdi>978-3131394835</bdi>. Archived from <a rel="nofollow" class="external text" href="https://www.lymphedemastore.com/ViewProducts.aspx?cid=106">the original</a> on 26 April 2014<span class="reference-accessdate">. Retrieved <span class="nowrap">25 April</span> 2014</span>.</cite></span>

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<li id="cite_note-3"><span class="mw-cite-backlink"><b><a href="#cite_ref-3">^</a></b></span> <span class="reference-text"><cite id="CITEREFChungKimJeffers2000" class="citation journal cs1">Chung, Kevin C.; Kim, Huyi Jin Elizabeth; Jeffers, Lynn L. C. (2000-11-01). <span class="id-lock-subscription" title="Paid subscription required"><a rel="nofollow" class="external text" href="https://www.sciencedirect.com/science/article/pii/S036350230065102X">"Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients"</a></span>. <i>The Journal of Hand Surgery</i>. <b>25</b> (6): <span class="nowrap">1163–</span>1168. <a href="Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1053%2Fjhsu.2000.18490">10.1053/jhsu.2000.18490</a>. <a href="ISSN_(identifier)" class="mw-redirect" title="ISSN (identifier)">ISSN</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/issn/0363-5023">0363-5023</a>. <a href="PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/11119680">11119680</a>.</cite></span>

</li>

<li id="cite_note-4"><span class="mw-cite-backlink"><b><a href="#cite_ref-4">^</a></b></span> <span class="reference-text">Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.</span>

</li>

<li id="cite_note-5"><span class="mw-cite-backlink"><b><a href="#cite_ref-5">^</a></b></span> <span class="reference-text">Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310–313</span>

</li>

<li id="cite_note-6"><span class="mw-cite-backlink"><b><a href="#cite_ref-6">^</a></b></span> <span class="reference-text">Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves Syndrome: a lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.</span>

</li>

<li id="cite_note-7"><span class="mw-cite-backlink"><b><a href="#cite_ref-7">^</a></b></span> <span class="reference-text">Gambini D, Visintin R, Locatelli E, Galassi B, Bareggi C, Runza L, Onida F, Tomirotti M.Tumori. 2009 Nov–Dec;95(6):828–31.

Paclitaxel-dependent prolonged and persistent complete remission four years from first recurrence of secondary breast angiosarcoma. Medical Oncology Unit, IRCCS Foundation "Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena", Milan, Italy.</span>

</li>

<li id="cite_note-8"><span class="mw-cite-backlink"><b><a href="#cite_ref-8">^</a></b></span> <span class="reference-text">Verdier E, Carvalho P, Young P, Musette P, Courville P, Joly P. Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)] Ann Dermatol Venereol. 2007 Oct;134 (10 Pt 1):760–3. French.</span>

</li>

<li id="cite_note-9"><span class="mw-cite-backlink"><b><a href="#cite_ref-9">^</a></b></span> <span class="reference-text">Sher T, Hennessy BT, Valero V, Broglio K, Woodward WA, Trent J, Hunt KK, Hortobagyi GN, Gonzalez-Angulo AM.Primary angiosarcomas of the breast. Cancer. 2007 Jul 1;110(1):173–8.</span>

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<div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2></div>

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</style></div><div role="navigation" class="navbox" aria-label="Navbox32" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q3913024" class="extiw external" title="d:Q3913024">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="International_Classification_of_Diseases_for_Oncology" title="International Classification of Diseases for Oncology">ICD-O</a></b>: 9170/3</li><li><b><a href="Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D008204">D008204</a></li><li><b><a href="Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb29095.htm">29095</a></li></ul></div></div></td></tr></tbody></table></div>

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</style><div id="Tumours_of_blood_vessels101" style="font-size:114%;margin:0 4em"><a href="Vascular_tumor" title="Vascular tumor">Tumours of blood vessels</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="Blood_vessel" title="Blood vessel">Blood vessel</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em">

<ul><li><a href="Hemangiosarcoma" title="Hemangiosarcoma">Hemangiosarcoma</a></li>

<li><a href="Blue_rubber_bleb_nevus_syndrome" title="Blue rubber bleb nevus syndrome">Blue rubber bleb nevus syndrome</a></li>

<li><a href="Hemangioendothelioma" title="Hemangioendothelioma">Hemangioendothelioma</a>

<ul><li><a href="Composite_hemangioendothelioma" class="mw-redirect" title="Composite hemangioendothelioma">Composite</a></li>

<li><a href="Endovascular_papillary_hemangioendothelioma" class="mw-redirect" title="Endovascular papillary hemangioendothelioma">Endovascular papillary</a></li>

<li><a href="Epithelioid_hemangioendothelioma" title="Epithelioid hemangioendothelioma">Epithelioid</a></li>

<li><a href="Kaposiform_hemangioendothelioma" class="mw-redirect" title="Kaposiform hemangioendothelioma">Kaposiform</a></li>

<li><a href="Infantile_hemangioendothelioma" class="mw-redirect" title="Infantile hemangioendothelioma">Infantile</a></li>

<li><a href="Retiform_hemangioendothelioma" class="mw-redirect" title="Retiform hemangioendothelioma">Retiform</a>)</li>

<li><a href="Spindle_cell_hemangioendothelioma" class="mw-redirect" title="Spindle cell hemangioendothelioma">Spindle cell</a></li></ul></li>

<li><a href="Proliferating_angioendotheliomatosis" title="Proliferating angioendotheliomatosis">Proliferating angioendotheliomatosis</a></li>

<li><a href="Hemangiopericytoma" title="Hemangiopericytoma">Hemangiopericytoma</a>

<ul><li><a href="Infantile_hemangiopericytoma" title="Infantile hemangiopericytoma">Infantile hemangiopericytoma</a></li></ul></li>

<li><a href="Venous_lake" title="Venous lake">Venous lake</a></li>

<li><a href="Kaposi's_sarcoma" title="Kaposi's sarcoma">Kaposi's sarcoma</a>

<ul><li><a href="African_cutaneous_Kaposi_sarcoma" class="mw-redirect" title="African cutaneous Kaposi sarcoma">African cutaneous</a></li>

<li><a href="African_lymphadenopathic_Kaposi_sarcoma" class="mw-redirect" title="African lymphadenopathic Kaposi sarcoma">African lymphadenopathic</a></li>

<li><a href="AIDS-associated_Kaposi_sarcoma" class="mw-redirect" title="AIDS-associated Kaposi sarcoma">AIDS-associated</a></li>

<li><a href="Classic_Kaposi_sarcoma" class="mw-redirect" title="Classic Kaposi sarcoma">Classic</a></li>

<li><a href="Immunosuppression-associated_Kaposi_sarcoma" class="mw-redirect" title="Immunosuppression-associated Kaposi sarcoma">Immunosuppression-associated</a></li></ul></li>

<li><a href="Hemangioblastoma" title="Hemangioblastoma">Hemangioblastoma</a></li>

<li><a href="Hemangioma" title="Hemangioma">Hemangioma</a>

<ul><li><a href="Capillary_hemangioma" class="mw-redirect" title="Capillary hemangioma">Capillary</a></li>

<li><a href="Cavernous_hemangioma" title="Cavernous hemangioma">Cavernous</a></li>

<li><a href="Glomeruloid_hemangioma" title="Glomeruloid hemangioma">Glomeruloid</a></li>

<li><a href="Microvenular_hemangioma" title="Microvenular hemangioma">Microvenular</a></li>

<li><a href="Targetoid_hemosiderotic_hemangioma" title="Targetoid hemosiderotic hemangioma">Targeted hemosiderotic</a></li></ul></li>

<li><a href="Angioma" title="Angioma">Angioma</a>

<ul><li><a href="Cherry_angioma" title="Cherry angioma">Cherry</a></li>

<li><a href="Angioma_serpiginosum" title="Angioma serpiginosum">Seriginosum</a></li>

<li><a href="Spider_angioma" title="Spider angioma">Spider</a></li>

<li><a href="Tufted_angioma" title="Tufted angioma">Tufted</a></li></ul></li>

<li>Universal angiomatosis</li>

<li><a href="Angiokeratoma" title="Angiokeratoma">Angiokeratoma</a>

<ul><li><a href="Angiokeratoma_of_Mibelli" class="mw-redirect" title="Angiokeratoma of Mibelli">of Mibelli</a></li>

<li><a href="Angiolipoma" title="Angiolipoma">Angiolipoma</a></li></ul></li>

<li><a href="Pyogenic_granuloma" title="Pyogenic granuloma">Pyogenic granuloma</a></li>

<li><a href="Intravascular_papillary_endothelial_hyperplasia" title="Intravascular papillary endothelial hyperplasia">Intravascular papillary endothelial hyperplasia</a></li></ul>

</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="Lymphatic_vessel_tumor" class="mw-redirect" title="Lymphatic vessel tumor">Lymphatic</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em">

<ul><li><a href="Lymphangioma" class="mw-redirect" title="Lymphangioma">Lymphangioma</a>/

<ul><li><a href="Lymphangioma_circumscriptum" title="Lymphangioma circumscriptum">Lymphangioma circumscriptum</a></li>

<li><a href="Acquired_progressive_lymphangioma" title="Acquired progressive lymphangioma">Acquired progressive lymphangioma</a></li></ul></li>

<li><i><a href="PEComa" class="mw-redirect" title="PEComa">PEComa</a></i>

<ul><li><a href="Lymphangioleiomyomatosis" title="Lymphangioleiomyomatosis">Lymphangioleiomyomatosis</a></li></ul></li>

<li><a href="Cystic_hygroma" title="Cystic hygroma">Cystic hygroma</a></li>

<li><a href="Multifocal_lymphangioendotheliomatosis" title="Multifocal lymphangioendotheliomatosis">Multifocal lymphangioendotheliomatosis</a></li>

<li><a href="Lymphangiomatosis" title="Lymphangiomatosis">Lymphangiomatosis</a></li></ul>

</div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Either</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em">

<ul><li><a href="Angioma" title="Angioma">Angioma</a>/<a href="Angiosarcoma" title="Angiosarcoma">angiosarcoma</a></li>

<li><a href="Angiofibroma" title="Angiofibroma">Angiofibroma</a></li></ul>

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This article is issued from <a class="external text" title="Last edited on 2025-05-24" href="https://en.wikipedia.org/wiki/?title=Lymphangiosarcoma&oldid=1291890782">Wikipedia</a>. The text is available under <a class="external text" href="https://creativecommons.org/licenses/by-sa/4.0/deed.en">Creative Commons Attribution-Share Alike 4.0</a> unless otherwise noted. Additional terms may apply for the media files.

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