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Angiosarcoma
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<h2 id="classification">Classification</h2> <p>The classification of angiosarcoma is based on both its origin and the underlying <a href="/facts/Risk_factor/N0YTUyer">risk factors</a> that may have contributed.<a class="footnote-ref" id="fnref:17" href="#fn:17"><sup>17</sup></a> This is not a comprehensive list and case reports often go into further depth.<a class="footnote-ref" id="fnref:18" href="#fn:18"><sup>18</sup></a> </p> <h3>Primary cutaneous angiosarcoma</h3> <p>Angiosarcoma in the <a href="/facts/Human_skin/cfRXQwSs">skin</a> (usually the <a href="/facts/Human_head/5ydPfI0C">head</a> or <a href="/facts/Neck/v69tnMYk">neck</a> and specifically the <a href="/facts/Scalp/yfnQt0n5">scalp</a>) with no underlying risk factors such as <a href="/facts/Lymphedema/BLCUBCvY">lymphedema</a> or <a href="/facts/Radiation_therapy/j60q5EC6">radiation therapy</a>. </p> <h3>Lymphedema associated angiosarcoma</h3> <p>Also called <a href="/facts/Stewart%25E2%2580%2593Treves_syndrome/dlD5hps1">Stewart-Treves syndrome</a>, it is a <a href="/facts/Lymphangiosarcoma/T0tF9Knb">lymphangiosarcoma</a> resulting from chronic <a href="/facts/Lymphedema/BLCUBCvY">lymphedema</a>, swelling due to the build up of <a href="/facts/Lymph/SL1xvRhq">lymphatic fluid</a>. It typically happens in the breast following a <a href="/facts/Mastectomy/hRAcK3at">mastectomy</a> that removes the <a href="/facts/Lymph_node/vkwfcQjH">lymph nodes</a> of the breast. However it can happen anywhere.<a class="footnote-ref" id="fnref:19" href="#fn:19"><sup>19</sup></a><a class="footnote-ref" id="fnref:20" href="#fn:20"><sup>20</sup></a> </p> <h3>Parenchymal angiosarcoma</h3> <p>Angiosarcoma in <a href="/facts/Parenchyma/NlGkFWlQ">parenchymal organs</a> such as the <a href="/facts/Liver/Rtp4Fk2b">liver</a>, <a href="/facts/Breast/UBvcKAjy">breast</a> or <a href="/facts/Heart/fF4KSGdk">heart</a>. These makes up about 40% of angiosarcoma. </p> <h4>Primary breast angiosarcoma</h4> <p>This refers to angiosarcoma of the breast <a href="/facts/Idiopathic_disease/YIVAonwA">without underlying causes</a> such as <a href="/facts/Radiation_therapy/j60q5EC6">radiation therapy</a> or <a href="/facts/Lymphedema/BLCUBCvY">lymphedema</a>. It is rare and makes up only 0.04% of <a href="/facts/Breast_cancer/6hME580v">breast tumors</a> and 8% of breast <a href="/facts/Sarcoma/F5kKDAGx">sarcomas</a>. It is very aggressive, usually presents in younger females and is often noticeable by a <a href="/facts/Palpation/6tnvdcJY">palpable</a> mass. Prognosis is poor with a five-year survival between 8 and 50%.<a class="footnote-ref" id="fnref:21" href="#fn:21"><sup>21</sup></a> </p> <h4>Secondary breast angiosarcoma</h4> <p>These are angiosarcoma that arise in the <a href="/facts/Breast/UBvcKAjy">breast</a> from <a href="/facts/Risk_factor/N0YTUyer">underlying causes</a> such as <a href="/facts/Lymphedema/BLCUBCvY">lymphedema</a> or radiation, usually from <a href="/facts/Radiation_therapy/j60q5EC6">radiation therapy</a> for <a href="/facts/Breast_cancer/6hME580v">breast cancer</a>. The link between radiation therapy and angiosarcoma has been disputed by some who profess the angiosarcoma is caused by concurrent lymphedema. Secondary breast angiosarcoma tends to have a bad prognosis. Radiation-associated angiosarcoma has a five-year survival of 10 to 54%. Distant <a href="/facts/Metastasis/4dadbm6z">metastases</a> are present in 27–42% of individuals and is associated with a far worse prognosis. Other prognostic factors include size of tumor, <a href="/facts/Ageing/AxL4qCW4">age</a> and how much can be removed by surgery.<a class="footnote-ref" id="fnref:22" href="#fn:22"><sup>22</sup></a><a class="footnote-ref" id="fnref:23" href="#fn:23"><sup>23</sup></a> </p> <h4>Primary brain angiosarcoma</h4> <p>Primary brain angiosarcoma are angiosarcoma that arise in the brain (i.e. not metastasised from elsewhere). They are exceedingly rare with only a few cases reported. Imaging is non specific and differential diagnosis includes most other brain tumors such as <a href="/facts/Glioma/I2gN3x11">gliomas</a> or <a href="/facts/Cavernous_hemangioma/phPh8dKW">cavernomas</a>,<a class="footnote-ref" id="fnref:24" href="#fn:24"><sup>24</sup></a> requiring biopsy to confirm diagnosis, usually after surgery. Prognosis is generally poor with a median survival rate of eight months. However it varies greatly depending on whether or not the tumor has metastasised, some individuals may be rid of the disease following surgery, chemotherapy and radiation therapy.<a class="footnote-ref" id="fnref:25" href="#fn:25"><sup>25</sup></a> </p> <h4>Primary cardiac angiosarcoma</h4> <p>Primary cardiac angiosarcoma is an angiosarcoma that arises in the <a href="/facts/Heart/fF4KSGdk">heart</a>. Despite being very rare they are the most common malignant <a href="/facts/Primary_tumors_of_the_heart/g2MGJ6rb">primary heart tumor</a> with 10–25%<a class="footnote-ref" id="fnref:26" href="#fn:26"><sup>26</sup></a><a class="footnote-ref" id="fnref:27" href="#fn:27"><sup>27</sup></a> of cases being angiosarcomas. Symptoms may include shortness of breath, chest pain (46%<a class="footnote-ref" id="fnref:28" href="#fn:28"><sup>28</sup></a>), <a href="/facts/Hypotension/BhrdVVRS">hypotension</a> and <a href="/facts/Syncope_(medicine)/AqU1whYh">syncope</a>. <a href="/facts/Superior_vena_cava_syndrome/sYf9AaPM">Superior vena cava syndrome</a> is reportedly a <a href="/facts/Complication_(medicine)/uJHvzqUt">complication</a> of cardiac angiosarcoma.<a class="footnote-ref" id="fnref:29" href="#fn:29"><sup>29</sup></a> Due to the non-specificity of symptoms and rarity of the disease it is often missed by doctors and initial diagnosis may be delayed. A 2012 study reported that 56% of patients presented with <a href="/facts/Pericardial_effusion/JHybCM7I">pericardial effusion</a> with or without <a href="/facts/Cardiac_tamponade/kfzbVAw7">cardiac tamponade</a>.<a class="footnote-ref" id="fnref:30" href="#fn:30"><sup>30</sup></a> The most common finding on <a href="/facts/Medical_imaging/pL6JyJEp">imaging</a> is <a href="/facts/Cardiomegaly/rYLRQ3C4">cardiomegaly</a>. Prognosis is generally very poor with a mean survival rate of three months to four years following diagnosis.<a class="footnote-ref" id="fnref:31" href="#fn:31"><sup>31</sup></a> Metastasis at time of diagnosis is common.<a class="footnote-ref" id="fnref:32" href="#fn:32"><sup>32</sup></a> </p> <h4>Primary liver angiosarcoma</h4> <p class="note">Main article: <a href="/facts/Liver_angiosarcoma/IsQMwKR8">Liver angiosarcoma</a></p> <p>Primary angiosarcoma of the liver is the third most common type of <a href="/facts/Liver_cancer/a7N4vD4L">liver cancer</a> however it only makes up 0.1–2.0% of all liver cancers. It is rapidly fatal with most individuals dying within six months or a year even with <a href="/facts/Segmental_resection/Lsvle5Cs">surgical resection</a>. Despite being associated with <a href="/facts/Arsenic/k1c4f44D">arsenic</a>, <a href="/facts/Vinyl_chloride/UNGUdwTK">vinyl chloride</a>, <a href="/facts/Thorotrast/VFQ19JKa">thorotrast</a> and other chemicals, 75% of liver angiosarcomas have <a href="/facts/Idiopathic_disease/YIVAonwA">no known cause</a>. Men are more commonly affected in a ratio of 3–4:1 however in children girls are more often affected. If symptomatic it often presents with non-specific liver related symptoms such as <a href="/facts/Abdominal_pain/aF6gPB0G">abdominal pain</a>, <a href="/facts/Jaundice/TCyoLMJU">jaundice</a>, <a href="/facts/Fatigue/C2oo4Hvg">fatigue</a>, <a href="/facts/Weight_loss/DQE4cXw0">unintended weight loss</a> and <a href="/facts/Abdominal_distension/EV3Rv672">distension</a>.<a class="footnote-ref" id="fnref:33" href="#fn:33"><sup>33</sup></a> </p> <h3>Deep soft tissue angiosarcoma</h3> <p>Angiosarcoma in the deep <a href="/facts/Soft_tissue/cHBwqLpP">soft tissue</a> such as <a href="/facts/Adipose_tissue/PxxedB6e">adipose tissue</a>, <a href="/facts/Ligament/xAf6lB1z">ligaments</a> or <a href="/facts/Skeletal_muscle/en1tVYFN">muscles</a>. These make up about 10% of angiosarcoma. </p> <h2 id="signs-and-symptoms">Signs and symptoms</h2> <p>Cutaneous angiosarcoma may initially look like a <a href="/facts/Bruise/ohkEH5BX">bruise</a> or a purplish-red <a href="/facts/Papule/2J7QwJdE">papule</a>. It may look like a <a href="/facts/Benign_tumor/ssJPkY84">benign tumor</a> which can delay correct diagnosis and treatment. <a href="/facts/Fungating_lesion/vdAe5uAJ">Fungation</a>, <a href="/facts/Ulcer_(dermatology)/ALY0IW1n">ulceration</a> and <a href="/facts/Bleeding/CGyFMU0F">bleeding</a> may develop.<a class="footnote-ref" id="fnref:34" href="#fn:34"><sup>34</sup></a> </p><p>Cardiac angiosarcoma may present with <a href="/facts/Shortness_of_breath/1jBfFzxU">shortness of breath</a>, <a href="/facts/Chest_pain/QFUzPBsT">chest pain</a> (46%), <a href="/facts/Unintentional_weight_loss/DQE4cXw0">weight loss</a>, <a href="/facts/Anemia/AgEPPmg8">anemia</a>-like symptoms,<a class="footnote-ref" id="fnref:35" href="#fn:35"><sup>35</sup></a> <a href="/facts/Hypotension/BhrdVVRS">low blood pressure</a> and <a href="/facts/Syncope_(medicine)/AqU1whYh">syncope</a>.<a class="footnote-ref" id="fnref:36" href="#fn:36"><sup>36</sup></a> </p><p>Angiosarcomas principally metastasises through the blood with the most common site for metastasis being the lungs; this may present as <a href="/facts/Pleural_effusion/34Y7pyFv">pleural effusion</a>, <a href="/facts/Pneumothorax/oaCPSynh">pneumothorax</a> or other pleural disease. Other common sites of metastasis include the liver, bone and lymph nodes.<a class="footnote-ref" id="fnref:37" href="#fn:37"><sup>37</sup></a> </p> <h2 id="causes">Causes</h2> <p>Angiosarcoma develops when damage to the <a href="/facts/DNA/nB89Iauz">DNA</a> of <a href="/facts/Endothelium/L4ENFVnw">endothelial cells</a> causes them to <a href="/facts/Cell_proliferation/Jp3hWo2t">divide uncontrollably</a> and prevents <a href="/facts/Programmed_cell_death/DWWhHTKj">programmed death</a>. This allows them to grow into a <a href="/facts/Neoplasm/K0ytzmPz">mass</a> invading other parts of the body and consuming its resources. If part of the mass breaks off and enters the <a href="/facts/Circulatory_system/WXwudzzc">circulatory</a> or <a href="/facts/Lymphatic_system/yw5QLgMT">lymphatic system</a> (which is very common due to angiosarcomas arising from the <a href="/facts/Endothelium/L4ENFVnw">lining</a> of said systems) it allows them to lodge elsewhere in the body. This process is called <a href="/facts/Metastasis/4dadbm6z">metastasis</a> and is a negative prognostic factor.<a class="footnote-ref" id="fnref:38" href="#fn:38"><sup>38</sup></a> The underlying causes of DNA damage is not understood but several risk factors are widely accepted. </p><p>Accepted risk factors include <a href="/facts/Lymphedema/BLCUBCvY">lymphedema</a>, <a href="/facts/Radiation_therapy/j60q5EC6">radiation therapy</a>, <a href="/facts/Neurofibromatosis/2R3QvYfn">neurofibromatosis</a>, <a href="/facts/Maffucci_syndrome/2VeBU8A7">Maffucci syndrome</a>, <a href="/facts/Klippel%25E2%2580%2593Tr%25C3%25A9naunay_syndrome/Is5N8M3P">Klippel-Trenaunay syndrome</a><a class="footnote-ref" id="fnref:39" href="#fn:39"><sup>39</sup></a> and chemicals such as <a href="/facts/Arsenic/k1c4f44D">arsenic</a>, <a href="/facts/Thorotrast/VFQ19JKa">thorotrast</a> and <a href="/facts/Vinyl_chloride/UNGUdwTK">vinyl chloride</a>.<a class="footnote-ref" id="fnref:40" href="#fn:40"><sup>40</sup></a> </p><p>A 2020 study analyzing the <a href="/facts/Genome/31Sm08JT">genome</a> of cutaneous angiosarcomas suggests that <a href="/facts/Ultraviolet/kncBUqn5">ultraviolet radiation</a> may be a <a href="/facts/Carcinogen/soH8Y9CE">causative agent</a> of angiosarcomas.<a class="footnote-ref" id="fnref:41" href="#fn:41"><sup>41</sup></a> </p><p><a href="/facts/Immunosuppression/22GHUHkr">Immunosuppression</a> may play a role in the <a href="/facts/Pathogenesis/o9kHHK0U">pathogenesis</a> of angiosarcoma. There have been reports of angiosarcoma in <a href="/facts/Immunosuppression/22GHUHkr">immunosuppressed</a> individuals following <a href="/facts/Kidney_transplantation/WMeCmTBL">kidney transplantation</a>. The association between lymphedema and angiosarcoma may be due to localized <a href="/facts/Immunodeficiency/CIEujXmc">immunodeficiency</a>, however this <a href="/facts/Hypothesis/gMABEJul">hypothesis</a> has not been confirmed. Some studies suggest a link between <a href="/facts/HIV%2fAIDS/RqLIEMuJ">AIDS</a> and angiosarcoma though this may be due to <a href="/facts/Medical_error/1RyM0bdK">misdiagnosed</a> <a href="/facts/Kaposi%2527s_sarcoma/0IFdzpbH">Kaposi's sarcoma</a>.<a class="footnote-ref" id="fnref:42" href="#fn:42"><sup>42</sup></a> </p> <h2 id="diagnosis">Diagnosis</h2> <p>The diagnosis of angiosarcoma can be difficult due to its asymptomatic nature or non-specific symptoms. Initial diagnosis is typically done by <a href="/facts/Magnetic_resonance_imaging/Vh9yG9qq">MRI</a>, <a href="/facts/CT_scan/NusPJ2S8">CT</a> or <a href="/facts/Medical_ultrasound/Ju5BFuuV">ultrasound</a> scan, however it is typically difficult to discern if a <a href="/facts/Neoplasm/K0ytzmPz">mass</a> is an angiosarcoma or other type of tumor such as a <a href="/facts/Melanoma/qdT7E97Y">melanoma</a> or <a href="/facts/Carcinoma/NE9UY4VB">carcinoma</a>. This means that while the initial diagnosis is typically done via imaging, conclusive diagnosis has to be performed via biopsy and subsequent <a href="/facts/Histology/yrI90zlU">histological</a> and <a href="/facts/Immunohistochemistry/knPpmAFf">immunohistochemical analysis</a>.<a class="footnote-ref" id="fnref:43" href="#fn:43"><sup>43</sup></a> </p><p>The <a href="/facts/Tumor_marker/o1BnIxlV">marker</a> <a href="/facts/CD31/yMKqNHdm">CD31</a> is often considered the gold standard for angiosarcoma diagnosis due to its high sensitivity and specificity. Other <a href="/facts/Tumor_marker/o1BnIxlV">markers</a> typically used include <a href="/facts/Von_Willebrand_factor/64IHfEJ9">von Willebrand factor</a>, <a href="/facts/CD34/KgRvVr0m">CD34</a>, and <a href="/facts/Vascular_endothelial_growth_factor/kNDc0Xj0">vascular endothelial growth factor</a>.<a class="footnote-ref" id="fnref:44" href="#fn:44"><sup>44</sup></a> The absence of <a href="/facts/Tumor_marker/o1BnIxlV">markers</a> present in <a href="/facts/Melanoma/qdT7E97Y">melanomas</a> such as <a href="/facts/S100_protein/NEVdr8Ue">S100</a> and <a href="/facts/HMB-45/GeylSMA7">HMB-45</a> can help in differentiating angiosarcomas from <a href="/facts/Melanoma/qdT7E97Y">melanomas</a>.<a class="footnote-ref" id="fnref:45" href="#fn:45"><sup>45</sup></a> </p> <h2 id="epidemiology">Epidemiology</h2> <p>Angiosarcomas are rare with one in a million people being diagnosed with it each year in the US.<a class="footnote-ref" id="fnref:46" href="#fn:46"><sup>46</sup></a> Older adults are more commonly affected and there is no gender bias,<a class="footnote-ref" id="fnref:47" href="#fn:47"><sup>47</sup></a> except in angiosarcoma of the liver where males are affected in a ratio of 3-4:1<a class="footnote-ref" id="fnref:48" href="#fn:48"><sup>48</sup></a> and cutaneous angiosarcoma where males are again affected more commonly, particularly elderly white men.<a class="footnote-ref" id="fnref:49" href="#fn:49"><sup>49</sup></a><a class="footnote-ref" id="fnref:50" href="#fn:50"><sup>50</sup></a> </p> <h2 id="other-animals">Other animals</h2> <p>In dogs, <a href="/facts/Hemangiosarcoma/5C9lTlPe">hemangiosarcoma</a> is relatively common with the <a href="/facts/Golden_retriever/dhcrPPcD">golden retriever</a>, <a href="/facts/Boxer_(dog)/zT7CtjA7">boxer</a> and <a href="/facts/Labrador_Retriever/movBWo1w">labrador retriever</a> at higher risk of the disease than other breeds. It occurs primarily in the <a href="/facts/Spleen/6NKn9d8s">spleen</a>, <a href="/facts/Liver/Rtp4Fk2b">liver</a>, <a href="/facts/Heart/fF4KSGdk">heart</a>, <a href="/facts/Skin/4havlLzG">skin</a> and <a href="/facts/Subcutaneous_tissue/EPYjXN3G">subcutaneous layer of the skin</a>. It is rapidly fatal and <a href="/facts/Metastasis/4dadbm6z">metastatic</a> and can present with anything from <a href="/facts/Nonspecific_symptom/BzP6MrTN">nonspecific signs</a> to imminent death from tumor rupture.<a class="footnote-ref" id="fnref:51" href="#fn:51"><sup>51</sup></a> </p> <h2 id="see-also">See also</h2> <ul><li><a href="/facts/Glomangiosarcoma/RtAFrfsN">Glomangiosarcoma</a></li> <li><a href="/facts/List_of_cutaneous_conditions/U58kszg2">List of cutaneous conditions</a></li></ul> <h2 id="references">References</h2> <ol> <li id="fn:1"><p>Cao, Jun; Wang, Jiale; He, Chiyu; Fang, Meiyu (2019-11-01). "Angiosarcoma: a review of diagnosis and current treatment". American Journal of Cancer Research. 9 (11): 2303–2313. ISSN 2156-6976. PMC 6895451. PMID 31815036. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6895451" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6895451</a> <a href="#fnref:1" class="footnote-back-ref">↩</a></p></li> <li id="fn:2"><p>Zhang, Chao; Xu, Guijun; Liu, Zheng; Xu, Yao; Lin, Feng; Baklaushev, Vladimir P.; Chekhonin, Vladimir P.; Peltzer, Karl; Mao, Min; Wang, Xin; Wang, Guowen (2019-12-27). "Epidemiology, tumor characteristics and survival in patients with angiosarcoma in the United States: a population-based study of 4537 cases". Japanese Journal of Clinical Oncology. 49 (12): 1092–1099. doi:10.1093/jjco/hyz113. ISSN 1465-3621. PMID 31612920. <a href="https://doi.org/10.1093%2Fjjco%2Fhyz113" target="_blank">https://doi.org/10.1093%2Fjjco%2Fhyz113</a> <a href="#fnref:2" class="footnote-back-ref">↩</a></p></li> <li id="fn:3"><p>Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A, Broering G, Elsayes KM (July 2017). "Angiosarcoma: clinical and imaging features from head to toe". Br J Radiol. 90 (1075): 20170039. doi:10.1259/bjr.20170039. PMC 5594986. PMID 28471264. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594986" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594986</a> <a href="#fnref:3" class="footnote-back-ref">↩</a></p></li> <li id="fn:4"><p>Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A, Broering G, Elsayes KM (July 2017). "Angiosarcoma: clinical and imaging features from head to toe". Br J Radiol. 90 (1075): 20170039. doi:10.1259/bjr.20170039. PMC 5594986. PMID 28471264. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594986" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594986</a> <a href="#fnref:4" class="footnote-back-ref">↩</a></p></li> <li id="fn:5"><p>Hayman, J; Huygens, H (1983-05-01). "Angiosarcoma developing around a foreign body". Journal of Clinical Pathology. 36 (5): 515–518. doi:10.1136/jcp.36.5.515. ISSN 0021-9746. PMC 498277. PMID 6404947. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC498277" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC498277</a> <a href="#fnref:5" class="footnote-back-ref">↩</a></p></li> <li id="fn:6"><p>Schneider, Tim; Renney, John; Hayman, John (August 1997). "Angiosarcoma occurring with chronic osteomyelitis and residual foreign material. A case report of a late World War wound complication". ANZ Journal of Surgery. 67 (8): 576–578. doi:10.1111/j.1445-2197.1997.tb02045.x. ISSN 1445-1433. <a href="https://doi.org/10.1111%2Fj.1445-2197.1997.tb02045.x" target="_blank">https://doi.org/10.1111%2Fj.1445-2197.1997.tb02045.x</a> <a href="#fnref:6" class="footnote-back-ref">↩</a></p></li> <li id="fn:7"><p>Russo, Daniela; Campanino, Maria Raffaela; Cepurnaite, Rima; Gencarelli, Annarita; De Rosa, Filippo; Corvino, Antonio; Menkulazi, Marsela; Tammaro, Vincenzo; Fuggi, Mariano; Insabato, Luigi (December 2020). "Primary High-Grade Angiosarcoma of the Breast in a Young Woman With Breast Implants: A Rare Case and a Review of Literature". International Journal of Surgical Pathology. 28 (8): 906–912. doi:10.1177/1066896920924087. ISSN 1066-8969. 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