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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. See: This record Not genetically inherited (Orphanet)

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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

Angiosarcoma

Pathological process
- Disease
  - Disorder by Site
    - Connective and Soft Tissue Disorder
      - Connective and soft tissue neoplasm
        Sarcoma
        Angiosarcoma
        Adult Angiosarcoma
        Angiosarcoma Associated with Lymphedema
        Angiosarcoma of the Skin of the Arm after Radical Mastectomy followed by Lymphedema
        Angiosarcoma NCI Grade 2
        Angiosarcoma NCI Grade 3
        Bone angiosarcoma
        Conjunctival Angiosarcoma
        Conventional angiosarcoma
        Localized Angiosarcoma
        Metastatic angiosarcoma
        Advanced Angiosarcoma
        Locally Advanced Angiosarcoma
        Pediatric angiosarcoma
        Poorly Differentiated Angiosarcoma
        Radiation-Related Angiosarcoma
        Skin Radiation-Related Angiosarcoma
        Recurrent Angiosarcoma
        Recurrent Breast Angiosarcoma
        Recurrent Skin Angiosarcoma
        Soft Tissue Angiosarcoma
        Breast angiosarcoma
        Postradiation Breast Angiosarcoma
        Primary Breast Angiosarcoma
        Central nervous system angiosarcoma
        Penile Angiosarcoma
        Peripheral Intraneural Angiosarcoma
        Sinonasal Angiosarcoma
        Skin angiosarcoma
        Visceral Angiosarcoma
        Adrenal Gland Angiosarcoma
        Aorta angiosarcoma
        Bladder Angiosarcoma
        Cardiac Angiosarcoma
        Colorectal Angiosarcoma
        Gallbladder angiosarcoma
        Kidney Angiosarcoma
        Liver angiosarcoma
        Mediastinum angiosarcoma
        Ovarian angiosarcoma
        Pericardial Angiosarcoma
        Prostate angiosarcoma
        Pulmonary Artery Angiosarcoma
        Seminal Vesicle Angiosarcoma
        Small Intestinal Angiosarcoma
        Spleen angiosarcoma
        Superior vena cava angiosarcoma
        Thyroid gland angiosarcoma
        Uterine Angiosarcoma
        Vulvar Angiosarcoma
        Unresectable Angiosarcoma

Professional guidelines

PubMed

Management of Skin Sarcomas.

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

Management of Vascular Sarcoma.

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

Management of angiosarcoma.

Budd GT
Curr Oncol Rep 2002 Nov;4(6):515-9. doi: 10.1007/s11912-002-0066-3. PMID: 12354365

See all (181)

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

The genetics of vascular tumours: an update.

Torrence D, Antonescu CR
Histopathology 2022 Jan;80(1):19-32. doi: 10.1111/his.14458. PMID: 34958509 Free PMC Article

Cutaneous Sarcomas.

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

Cutaneous sarcomas.

Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457

Kutane Sarkome.

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

Angiosarcoma.

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

See all (1093)

Diagnosis

Cutaneous Sarcomas.

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

Primary Esophageal Angiosarcoma.

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

Radiation-induced angiosarcoma of the breast: A review.

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

Cutaneous Malignant Vascular Neoplasms.

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

Primary hepatic angiosarcoma.

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

See all (2729)

Therapy

Multicenter phase II trial (SWOG S1609, cohort 51) of ipilimumab and nivolumab in metastatic or unresectable angiosarcoma: a substudy of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART).

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: 34380663 Free PMC Article

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.

Requena C, Alsina M, Morgado-Carrasco D, Cruz J, Sanmartín O, Serra-Guillén C, Llombart B
Actas Dermosifiliogr (Engl Ed) 2018 Dec;109(10):878-887. Epub 2018 Sep 24 doi: 10.1016/j.ad.2018.06.013. PMID: 30262126

Vascular sarcomas.

Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636

Management of angiosarcoma.

Budd GT
Curr Oncol Rep 2002 Nov;4(6):515-9. doi: 10.1007/s11912-002-0066-3. PMID: 12354365

Radiation-induced sarcoma.

Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

See all (942)

Prognosis

Distinctive epidemiology and prognosis of de novo and secondary cutaneous angiosarcoma patients.

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

Sinonasal angiosarcoma.

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

Radiation-induced sarcoma.

Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168

Angiosarcoma: current review.

Fedok FG, Levin RJ, Maloney ME, Tipirneni K
Am J Otolaryngol 1999 Jul-Aug;20(4):223-31. doi: 10.1016/s0196-0709(99)90004-2. PMID: 10442774

Cardiac angiosarcoma.

Ohtahara A, Hattori K, Fukuki M, Hirata S, Ahmmed GU, Kato M, Fujimoto Y, Shigemasa C, Mashiba H
Intern Med 1996 Oct;35(10):795-8. doi: 10.2169/internalmedicine.35.795. PMID: 8933189

See all (1707)

Clinical prediction guides

Immunotherapy in Sarcoma: Where Do Things Stand?

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

Cutaneous angiosarcoma: a current update.

Shustef E, Kazlouskaya V, Prieto VG, Ivan D, Aung PP
J Clin Pathol 2017 Nov;70(11):917-925. Epub 2017 Sep 15 doi: 10.1136/jclinpath-2017-204601. PMID: 28916596

Vascular lesions of the breast.

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

Vascular sarcomas.

Ravi V, Patel S
Curr Oncol Rep 2013 Aug;15(4):347-55. doi: 10.1007/s11912-013-0328-2. PMID: 23852636

Angiosarcoma.

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

See all (620)

Recent systematic reviews

Treatment of Angiosarcoma of the Head and Neck: A Systematic Review.

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

Association between occupational exposures and sarcoma incidence and mortality: systematic review and meta-analysis.

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: 34389054 Free PMC Article

Primary thyroid angiosarcoma: A systematic review.

De Felice F, Moscatelli E, Orelli S, Bulzonetti N, Musio D, Tombolini V
Oral Oncol 2018 Jul;82:48-52. Epub 2018 May 11 doi: 10.1016/j.oraloncology.2018.05.004. PMID: 29909901

Predisposing factors for poor prognosis of angiosarcoma of the scalp and face: Systematic review and meta-analysis.

Shin JY, Roh SG, Lee NH, Yang KM
Head Neck 2017 Feb;39(2):380-386. Epub 2016 Aug 10 doi: 10.1002/hed.24554. PMID: 27507124

Non-hepatocellular carcinoma spinal metastases.

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

See all (37)

MedGen

National Center for Biotechnology Information

Send to:

Angiosarcoma

Definition

Term Hierarchy

Professional guidelines

PubMed

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Curated

Consumer resources

Reviews

Related information

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