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Angiosarcoma
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== Classification == The classification of angiosarcoma is based on both its origin and the underlying [[risk factor]]s that may have contributed.<ref name="headtotoe" /> This is not a comprehensive list and case reports often go into further depth.<ref name="Angiosarcoma">{{Cite journal|last1=Young|first1=Robin J.|last2=Brown|first2=Nicola J.|last3=Reed|first3=Malcolm W.|last4=Hughes|first4=David|last5=Woll|first5=Penella J.|date=October 2010|title=Angiosarcoma|url=https://pubmed.ncbi.nlm.nih.gov/20537949/|journal=The Lancet. Oncology|volume=11|issue=10|pages=983–991|doi=10.1016/S1470-2045(10)70023-1|issn=1474-5488|pmid=20537949}}</ref> === Primary cutaneous angiosarcoma === Angiosarcoma in the [[Human skin|skin]] (usually the [[Human head|head]] or [[neck]] and specifically the [[scalp]]) with no underlying risk factors such as [[lymphedema]] or [[radiation therapy]].{{citation needed|date=October 2021}} === Lymphedema associated angiosarcoma === Also called [[Stewart–Treves syndrome|Stewart-Treves syndrome]], it is a [[lymphangiosarcoma]] resulting from chronic [[lymphedema]], swelling due to the build up of [[Lymph|lymphatic fluid]]. It typically happens in the breast following a [[mastectomy]] that removes the [[lymph node]]s of the breast. However it can happen anywhere.<ref name="Angiosarcoma" /><ref name="headtotoe" /> === Parenchymal angiosarcoma === Angiosarcoma in [[Parenchyma|parenchymal organs]] such as the [[liver]], [[breast]] or [[heart]]. These makes up about 40% of angiosarcoma.{{citation needed|date=October 2021}} ==== Primary breast angiosarcoma ==== This refers to angiosarcoma of the breast [[Idiopathic disease|without underlying causes]] such as [[radiation therapy]] or [[lymphedema]]. It is rare and makes up only 0.04% of [[Breast cancer|breast tumors]] and 8% of breast [[sarcoma]]s. It is very aggressive, usually presents in younger females and is often noticeable by a [[Palpation|palpable]] mass. Prognosis is poor with a five-year survival between 8 and 50%.<ref name="headtotoe" /> ==== Secondary breast angiosarcoma ==== These are angiosarcoma that arise in the [[breast]] from [[Risk factor|underlying causes]] such as [[lymphedema]] or radiation, usually from [[radiation therapy]] for [[breast cancer]]. 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 [[Metastasis|metastases]] are present in 27–42% of individuals and is associated with a far worse prognosis. Other prognostic factors include size of tumor, [[Ageing|age]] and how much can be removed by surgery.<ref name="Angiosarcoma" /><ref name="headtotoe" /> ==== Primary brain angiosarcoma ==== 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 [[glioma]]s or [[Cavernous hemangioma|cavernomas]],<ref name="Angiosarcoma" /> 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.<ref>{{Cite journal|last1=La Corte|first1=Emanuele|last2=Acerbi|first2=Francesco|last3=Schiariti|first3=Marco|last4=Broggi|first4=Morgan|last5=Maderna|first5=Emanuela|last6=Pollo|first6=Bianca|last7=Nunziata|first7=Raffaele|last8=Maccagnano|first8=Elio|last9=Ferroli|first9=Paolo|date=April 2015|title=Primary central nervous system angiosarcoma: a case report and literature review|url=https://pubmed.ncbi.nlm.nih.gov/25388456/|journal=Neuropathology |volume=35|issue=2|pages=184–191|doi=10.1111/neup.12178|issn=1440-1789|pmid=25388456|s2cid=21199506}}</ref> ==== Primary cardiac angiosarcoma ==== Primary cardiac angiosarcoma is an angiosarcoma that arises in the [[heart]]. Despite being very rare they are the most common malignant [[Primary tumors of the heart|primary heart tumor]] with 10–25%<ref name=":2">{{Cite journal|last1=Patel|first1=Swetal Dilip|last2=Peterson|first2=Ashley|last3=Bartczak|first3=Artur|last4=Lee|first4=Sarah|last5=Chojnowski|first5=Sławomir|last6=Gajewski|first6=Piotr|last7=Loukas|first7=Marios|date=2014|title=Primary cardiac angiosarcoma – a review|journal=Medical Science Monitor|language=en|volume=20|pages=103–109|doi=10.12659/MSM.889875|pmid=24452054|pmc=3907509}}</ref><ref name="Angiosarcoma" /> of cases being angiosarcomas. Symptoms may include shortness of breath, chest pain (46%<ref name=":2" />), [[hypotension]] and [[Syncope (medicine)|syncope]]. [[Superior vena cava syndrome]] is reportedly a [[Complication (medicine)|complication]] of cardiac angiosarcoma.<ref name="headtotoe" /> 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 [[pericardial effusion]] with or without [[cardiac tamponade]].<ref name=":2" /> The most common finding on [[Medical imaging|imaging]] is [[cardiomegaly]]. Prognosis is generally very poor with a mean survival rate of three months to four years following diagnosis.<ref name="headtotoe" /> Metastasis at time of diagnosis is common.<ref name=":2" /> ==== Primary liver angiosarcoma ==== {{Main|Liver angiosarcoma}} Primary angiosarcoma of the liver is the third most common type of [[liver cancer]] 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 [[Segmental resection|surgical resection]]. Despite being associated with [[arsenic]], [[vinyl chloride]], [[thorotrast]] and other chemicals, 75% of liver angiosarcomas have [[Idiopathic disease|no known cause]]. 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 [[abdominal pain]], [[jaundice]], [[fatigue]], [[Weight loss|unintended weight loss]] and [[Abdominal distension|distension]].<ref name=":3" /> === Deep soft tissue angiosarcoma === Angiosarcoma in the deep [[soft tissue]] such as [[adipose tissue]], [[ligament]]s or [[Skeletal muscle|muscles]]. These make up about 10% of angiosarcoma.{{citation needed|date=October 2021}}
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