Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are more commonly affected, and the skin is the most affected area, with approximately 60% of cases being cutaneous (skin). Specifically, the scalp makes up ~50% of angiosarcoma cases, but this is still <0.1% of all head and neck tumors.[5][6][7] Since angiosarcoma is an 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 lesions, 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.[7]
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The cause of angiosarcoma is not known, though several risk factors 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.[8][9] Infrequently they have occurred in association with breast implants.[10] Ultraviolet radiation[2] and localized immunodeficiency may play a role in pathogenesis of angiosarcoma.[3] Angiosarcoma can be seen on MRI, CT and ultrasound scans, but it is usually difficult to discern it from other cancers, requiring confirmation of diagnosis by biopsy and immunohistochemical analysis.
Treatment includes surgery, chemotherapy and radiation therapy, usually all three combined. Because these cancers arise from the cells lining the blood or lymphatic vessels, they can easily metastasize to distant sites, particularly the liver and lungs.[7] 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 30–38%.[1][3] This is even worse in cardiac angiosarcoma and angiosarcoma of the liver, where prognosis may be as low as three months.[7]
Angiosarcomas make up 1–2% of soft tissue sarcomas, which in turn make up less than 1% of adult cancer.[2] Due to this, no large studies have ever been published on the disease, with few exceeding even 100 patients; however, many case reports and small cohort studies have been published, and they cumulatively provide enough information to get a useful understanding of the disease.[7] The rate of angiosarcoma is increasing in the US.[6]
The classification of angiosarcoma is based on both its origin and the underlying risk factors that may have contributed.[7] This is not a comprehensive list and case reports often go into further depth.[3]
Parenchymal angiosarcoma
Angiosarcoma in parenchymal organs such as the liver, breast or heart. These makes up about 40% of angiosarcoma.[citation needed]
Secondary breast angiosarcoma
These are angiosarcoma that arise in the breast from 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 metastases are present in 27–42% of individuals and is associated with a far worse prognosis. Other prognostic factors include size of tumor, age and how much can be removed by surgery.[3][7]
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 gliomas or cavernomas,[3] 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.[11]
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 heart tumor with 10–25%[12][3] of cases being angiosarcomas. Symptoms may include shortness of breath, chest pain (46%[12]), hypotension and syncope. Superior vena cava syndrome is reportedly a complication of cardiac angiosarcoma.[7] 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.[12] The most common finding on imaging is cardiomegaly. Prognosis is generally very poor with a mean survival rate of three months to four years following diagnosis.[7] Metastasis at time of diagnosis is common.[12]
Angiosarcomas are rare with one in a million people being diagnosed with it each year in the US.[4] Older adults are more commonly affected and there is no gender bias,[1] except in angiosarcoma of the liver where males are affected in a ratio of 3-4:1[13] and cutaneous angiosarcoma where males are again affected more commonly, particularly elderly white men.[3][7]
Kumar V, Abbas AK, Aster JC, eds. (2013). Robbins Basic Pathology (Ninth ed.). Elsevier/Saunders. pp. 361–362. ISBN 978-1437717815. Painter, Corrie A.; Jain, Esha; Tomson, Brett N.; Dunphy, Michael; Stoddard, Rachel E.; Thomas, Beena S.; Damon, Alyssa L.; Shah, Shahrayz; Kim, Dewey; Gómez Tejeda Zañudo, Jorge; Hornick, Jason L. (February 2020). "The Angiosarcoma Project: enabling genomic and clinical discoveries in a rare cancer through patient-partnered research". Nature Medicine. 26 (2): 181–187. doi:10.1038/s41591-019-0749-z. ISSN 1546-170X. PMID 32042194. S2CID 211074464. 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. 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. Kumar, Anila; Sharma, Bashar; Samant, Hrishikesh (2021), "Liver Angiosarcoma", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30855812, retrieved 2021-08-08