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Angiosarcoma

MedGen UID:
42385
Concept ID:
C0018923
Neoplastic Process
Synonyms: Angiosarcomas; Hemangiosarcoma; Hemangiosarcomas
SNOMED CT: Malignant hemangioendothelioma (33176006); Angiosarcoma (403977003); Angiosarcoma (863926008); Hemangioendothelioma, malignant (33176006); Hemangioendothelial sarcoma (33176006); Hemangiosarcoma (39000009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0200058
Monarch Initiative: MONDO:0016982
Orphanet: ORPHA263413

Definition

A rare vascular tumor characterized by a malignant space-occupying lesion composed of cells variably recapitulating features of normal endothelium. It mostly develops as a cutaneous tumor and is much less frequently located in the deep soft tissue. Clinical presentation is an enlarging mass, sometimes with symptoms like coagulopathy, anemia, persistent hematoma, or bruisability. Some tumors are associated with pre-existing conditions, e. g. Klippel-Trenaunay syndrome, Maffucci syndrome, or following radiation, among others. Older age, retroperitoneal location, large size, and high mitotic activity are predictors for poor outcome. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Angiosarcoma

Professional guidelines

PubMed

Houpe JE, Seger EW, Neill BC, Kang BY, Hocker TLH, Alam M, Tolkachjov SN
Cutis 2023 May;111(5):247-251. doi: 10.12788/cutis.0767. PMID: 37406332
Messina V, Cope B, Keung EZ, Fiore M
Surg Oncol Clin N Am 2022 Jul;31(3):511-525. Epub 2022 May 31 doi: 10.1016/j.soc.2022.03.010. PMID: 35715147
Subramaniam A, Giani C, Napolitano A, Ravi V, Frezza AM, Jones RL
Surg Oncol Clin N Am 2022 Jul;31(3):485-510. doi: 10.1016/j.soc.2022.03.014. PMID: 35715146

Recent clinical studies

Etiology

Torrence D, Antonescu CR
Histopathology 2022 Jan;80(1):19-32. doi: 10.1111/his.14458. PMID: 34958509Free PMC Article
Tillman BN, Liu JC
Otolaryngol Clin North Am 2021 Apr;54(2):369-378. Epub 2021 Feb 16 doi: 10.1016/j.otc.2020.11.010. PMID: 33602520
Bonito FJP, de Almeida Cerejeira D, Dahlstedt-Ferreira C, Oliveira Coelho H, Rosas R
Breast J 2020 Mar;26(3):458-463. Epub 2019 Aug 25 doi: 10.1111/tbj.13504. PMID: 31448482
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446
Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ
Lancet Oncol 2010 Oct;11(10):983-91. Epub 2010 May 25 doi: 10.1016/S1470-2045(10)70023-1. PMID: 20537949

Diagnosis

Tillman BN, Liu JC
Otolaryngol Clin North Am 2021 Apr;54(2):369-378. Epub 2021 Feb 16 doi: 10.1016/j.otc.2020.11.010. PMID: 33602520
Gutnik L, Goold E, Madsen J, Clayton F, Varghese T, Glasgow R
Ann Thorac Surg 2021 May;111(5):e365-e367. Epub 2020 Oct 20 doi: 10.1016/j.athoracsur.2020.07.087. PMID: 33096067
Bonito FJP, de Almeida Cerejeira D, Dahlstedt-Ferreira C, Oliveira Coelho H, Rosas R
Breast J 2020 Mar;26(3):458-463. Epub 2019 Aug 25 doi: 10.1111/tbj.13504. PMID: 31448482
Shon W, Billings SD
Clin Lab Med 2017 Sep;37(3):633-646. Epub 2017 Jul 13 doi: 10.1016/j.cll.2017.06.004. PMID: 28802504
Chaudhary P, Bhadana U, Singh RA, Ahuja A
Eur J Surg Oncol 2015 Sep;41(9):1137-43. Epub 2015 May 14 doi: 10.1016/j.ejso.2015.04.022. PMID: 26008857

Therapy

Wagner MJ, Othus M, Patel SP, Ryan C, Sangal A, Powers B, Budd GT, Victor AI, Hsueh CT, Chugh R, Nair S, Leu KM, Agulnik M, Sharon E, Mayerson E, Plets M, Blanke C, Streicher H, Chae YK, Kurzrock R
J Immunother Cancer 2021 Aug;9(8) doi: 10.1136/jitc-2021-002990. PMID: 34380663Free PMC Article
Florou V, Wilky BA
Curr Treat Options Oncol 2018 Mar 8;19(3):14. doi: 10.1007/s11864-018-0531-3. PMID: 29520447
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636
Penel N, Bui BN, Bay JO, Cupissol D, Ray-Coquard I, Piperno-Neumann S, Kerbrat P, Fournier C, Taieb S, Jimenez M, Isambert N, Peyrade F, Chevreau C, Bompas E, Brain EG, Blay JY
J Clin Oncol 2008 Nov 10;26(32):5269-74. Epub 2008 Sep 22 doi: 10.1200/JCO.2008.17.3146. PMID: 18809609
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

Prognosis

Ye J, Han W, Xu J
Int J Dermatol 2023 Jul;62(7):952-961. Epub 2023 Apr 17 doi: 10.1111/ijd.16673. PMID: 37066596
Wang X, Lu Z, Luo Y, Cai J, Wei J, Liu A, Zeng Z
Medicine (Baltimore) 2022 Feb 11;101(6):e28785. doi: 10.1097/MD.0000000000028785. PMID: 35147108Free PMC Article
Maduekwe UN, Herb JN, Esther RJ, Kim HJ, Spanheimer PM
J Surg Oncol 2021 May;123(8):1792-1800. Epub 2021 Mar 22 doi: 10.1002/jso.26465. PMID: 33751586Free PMC Article
Es-Sbissi F, Nitassi S, Boulaadas M, Essakalli L
Eur Ann Otorhinolaryngol Head Neck Dis 2015 Jun;132(3):161-3. Epub 2015 Feb 16 doi: 10.1016/j.anorl.2015.01.006. PMID: 25698587
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

Clinical prediction guides

Moreno Tellez C, Leyfman Y, D'Angelo SP, Wilky BA, Dufresne A
Surg Oncol Clin N Am 2022 Jul;31(3):381-397. doi: 10.1016/j.soc.2022.03.004. PMID: 35715140
Dajsakdipon T, Siripoon T, Ngamphaiboon N, Ativitavas T, Dejthevaporn T
Curr Treat Options Oncol 2022 Mar;23(3):415-438. Epub 2022 Mar 9 doi: 10.1007/s11864-022-00944-6. PMID: 35262852
Baker GM, Schnitt SJ
Semin Diagn Pathol 2017 Sep;34(5):410-419. Epub 2017 May 30 doi: 10.1053/j.semdp.2017.05.013. PMID: 28676174
Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636
Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ
Lancet Oncol 2010 Oct;11(10):983-91. Epub 2010 May 25 doi: 10.1016/S1470-2045(10)70023-1. PMID: 20537949

Recent systematic reviews

Houpe JE, Seger EW, Neill BC, Kang BY, Hocker TLH, Alam M, Tolkachjov SN
Cutis 2023 May;111(5):247-251. doi: 10.12788/cutis.0767. PMID: 37406332
Sato F, Yamamoto T
J Plast Reconstr Aesthet Surg 2022 Sep;75(9):2882-2889. Epub 2022 Jun 21 doi: 10.1016/j.bjps.2022.06.046. PMID: 35907689
Edwards D, Voronina A, Attwood K, Grand'Maison A
Syst Rev 2021 Aug 13;10(1):231. doi: 10.1186/s13643-021-01769-4. PMID: 34389054Free PMC Article
Abdou Y, Elkhanany A, Attwood K, Ji W, Takabe K, Opyrchal M
Breast Cancer Res Treat 2019 Dec;178(3):523-533. Epub 2019 Sep 14 doi: 10.1007/s10549-019-05432-4. PMID: 31522347Free PMC Article
Goodwin CR, Abu-Bonsrah N, Boone C, Ruiz-Valls A, Sankey EW, Sarabia-Estrada R, Elder BD, Kosztowski T, Sciubba DM
J Clin Neurosci 2016 May;27:22-7. Epub 2016 Jan 8 doi: 10.1016/j.jocn.2015.11.003. PMID: 26778049

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