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Angiosarcoma
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{{Short description|Cancer of the lining of the blood or lymphatic vessels}} {{Infobox medical condition (new) | name = Angiosarcoma | synonyms = | image = Metastatic angiosarcoma in the brain.jpg | alt = | caption = [[MRI]] of a cardiac angiosarcoma that has metastasised to the brain. | pronounce = | field = [[Oncology]] | symptoms = Often [[asymptomatic]], non-specific symptoms common | complications = | onset = Typically 60-70 however all age groups are affected | duration = | types = [[Hemangiosarcoma]], [[lymphangiosarcoma]], parenchymal angiosarcoma | causes = Unknown | risks = [[Lymphedema]], [[radiation therapy]], various chemicals | diagnosis = [[Imaging]], [[biopsy]] | differential = | prevention = | treatment = [[Chemotherapy]], [[radiation therapy]], surgery | medication = | prognosis = Depends on type, overall [[five-year survival]] ~ 30-38%<ref name ="robbins">{{Cite book|title=Robbins Basic Pathology|publisher=Elsevier/Saunders|year=2013|isbn=978-1437717815|veditors=Kumar V, Abbas AK, Aster JC|edition=Ninth|pages=361β362}}</ref><ref name="project" /><ref name="Angiosarcoma" /> | frequency = 1 per million people (US)<ref name="cancer.gov">{{Cite web|date=2019-02-27|title=Angiosarcoma - National Cancer Institute|url=https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-vascular-tumors/angiosarcoma|access-date=2021-08-08|website=www.cancer.gov|language=en}}</ref> | deaths = }} '''Angiosarcoma''' is a rare and aggressive [[cancer]] that starts in the [[endothelial]] cells that line the walls of [[blood vessel]]s or [[lymphatic vessel]]s. Since they are made from [[Endothelium|vascular lining]], they can appear anywhere and at any age, but older people are more commonly affected, and the [[Human skin|skin]] is the most affected area, with approximately 60% of cases being [[wikt:cutaneous|cutaneous]] (skin). Specifically, the [[scalp]] makes up ~50% of angiosarcoma cases, but this is still <0.1% of all head and neck tumors.<ref name=":0">{{Cite journal|last1=Cao|first1=Jun|last2=Wang|first2=Jiale|last3=He|first3=Chiyu|last4=Fang|first4=Meiyu|date=2019-11-01|title=Angiosarcoma: a review of diagnosis and current treatment|journal=American Journal of Cancer Research|volume=9|issue=11|pages=2303β2313|issn=2156-6976|pmc=6895451|pmid=31815036}}</ref><ref name=":1">{{Cite journal|last1=Zhang|first1=Chao|last2=Xu|first2=Guijun|last3=Liu|first3=Zheng|last4=Xu|first4=Yao|last5=Lin|first5=Feng|last6=Baklaushev|first6=Vladimir P.|last7=Chekhonin|first7=Vladimir P.|last8=Peltzer|first8=Karl|last9=Mao|first9=Min|last10=Wang|first10=Xin|last11=Wang|first11=Guowen|date=2019-12-27|title=Epidemiology, tumor characteristics and survival in patients with angiosarcoma in the United States: a population-based study of 4537 cases|journal=Japanese Journal of Clinical Oncology|volume=49|issue=12|pages=1092β1099|doi=10.1093/jjco/hyz113|issn=1465-3621|pmid=31612920|doi-access=free}}</ref><ref name="headtotoe" /> Since angiosarcoma is an [[Hyponymy and hypernymy|umbrella term]] for many types of tumor that vary greatly in origin and location, many symptoms may occur, from completely asymptomatic to non-specific symptoms like [[Skin condition|skin lesions]], [[Ulcer (dermatology)|ulceration]], [[shortness of breath]] and [[abdominal pain]]. Multiple-organ involvement at time of diagnosis is common and makes it difficult to ascertain origin and how to treat it.<ref name="headtotoe" /> The cause of angiosarcoma is not known, though several [[risk factor]]s are known, such as chronic [[lymphedema]], [[radiation therapy]] and various chemicals such as [[arsenic]] and [[vinyl chloride]]. Angiosarcomas have been reported in association with long standing foreign bodies.<ref>{{Cite journal |last1=Hayman |first1=J |last2=Huygens |first2=H |date=1983-05-01 |title=Angiosarcoma developing around a foreign body. |journal=Journal of Clinical Pathology |language=en |volume=36 |issue=5 |pages=515β518 |doi=10.1136/jcp.36.5.515 |issn=0021-9746 |pmc=498277 |pmid=6404947}}</ref><ref>{{Cite journal |last1=Schneider |first1=Tim |last2=Renney |first2=John |last3=Hayman |first3=John |date=August 1997 |title=Angiosarcoma occurring with chronic osteomyelitis and residual foreign material. A case report of a late World War wound complication. |journal=ANZ Journal of Surgery |language=en |volume=67 |issue=8 |pages=576β578 |doi=10.1111/j.1445-2197.1997.tb02045.x |issn=1445-1433|doi-access=free }}</ref> Infrequently they have occurred in association with breast implants.<ref>{{Cite journal |last1=Russo |first1=Daniela |last2=Campanino |first2=Maria Raffaela |last3=Cepurnaite |first3=Rima |last4=Gencarelli |first4=Annarita |last5=De Rosa |first5=Filippo |last6=Corvino |first6=Antonio |last7=Menkulazi |first7=Marsela |last8=Tammaro |first8=Vincenzo |last9=Fuggi |first9=Mariano |last10=Insabato |first10=Luigi |date=December 2020 |title=Primary High-Grade Angiosarcoma of the Breast in a Young Woman With Breast Implants: A Rare Case and a Review of Literature |url=http://journals.sagepub.com/doi/10.1177/1066896920924087 |journal=International Journal of Surgical Pathology |language=en |volume=28 |issue=8 |pages=906β912 |doi=10.1177/1066896920924087 |pmid=32423273 |issn=1066-8969|url-access=subscription }}</ref> [[Ultraviolet|Ultraviolet radiation]]<ref name="project" /> and [[Immunodeficiency|localized immunodeficiency]] may play a role in [[pathogenesis]] of angiosarcoma.<ref name="Angiosarcoma" /> Angiosarcoma can be seen on [[Magnetic resonance imaging|MRI]], [[CT scan|CT]] and [[Medical ultrasound|ultrasound]] scans, but it is usually difficult to discern it from other cancers, requiring confirmation of diagnosis by [[biopsy]] and [[Immunohistochemistry|immunohistochemical analysis]]. Treatment includes [[Surgical oncology|surgery]], [[chemotherapy]] and [[radiation therapy]], usually all three combined. Because these cancers arise from the [[Endothelium|cells lining the blood or lymphatic vessels]], they can easily [[Metastasis|metastasize]] to distant sites, particularly the [[liver]] and [[lung]]s.<ref name="headtotoe">{{cite journal|vauthors=Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A, Broering G, Elsayes KM|date=July 2017|title=Angiosarcoma: clinical and imaging features from head to toe|journal=Br J Radiol|volume=90|issue=1075|pages=20170039|doi=10.1259/bjr.20170039|pmc=5594986|pmid=28471264}}</ref> This makes them especially lethal, and an early diagnosis is usually necessary for survival. Even with treatment, prognosis is poor, with a [[five-year survival rate]] of 30β38%.<ref name="robbins" /><ref name="Angiosarcoma" /> This is even worse in cardiac angiosarcoma and angiosarcoma of the liver, where [[prognosis]] may be as low as three months.<ref name="headtotoe" /> Angiosarcomas make up 1β2% of [[Soft-tissue sarcoma|soft tissue sarcomas]], which in turn make up less than 1% of adult cancer.<ref name="project" /> Due to this, no large studies have ever been published on the disease, with few exceeding even 100 patients; however, many [[case report]]s and small [[Cohort study|cohort studies]] have been published, and they cumulatively provide enough information to get a useful understanding of the disease.<ref name="headtotoe" /> The rate of angiosarcoma is increasing in the US.<ref name=":1" />
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