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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

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

Professional guidelines

PubMed

Treatment of a primary cardiac angiosarcoma with stereotactic body radiation therapy.
Stergioula A, Pantelis E
Hellenic J Cardiol 2023 Jul-Aug;72:70-71. Epub 2023 Apr 7 doi: 10.1016/j.hjc.2023.04.001. PMID: 37030628
Primary angiosarcoma of the heart: The clinical presentation and management.
Xie J, Qin Y, Liu J
Asian J Surg 2023 Aug;46(8):3199-3200. Epub 2023 Mar 4 doi: 10.1016/j.asjsur.2023.02.117. PMID: 36878790
Cutaneous angiosarcoma: Efficacy and treatment regimen of paclitaxel maintenance therapy.
Saito Y, Yokoyama R, Umemori Y
Dermatol Ther 2020 Jul;33(4):e13563. Epub 2020 May 28 doi: 10.1111/dth.13563. PMID: 32401427

Curated

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

Recent clinical studies

Etiology

Cutaneous Mesenchymal Sarcomas.
Walocko F, Christensen RE, Worley B, Alam M
Dermatol Clin 2023 Jan;41(1):133-140. Epub 2022 Oct 28 doi: 10.1016/j.det.2022.07.011. PMID: 36410974
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
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
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
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

Diagnosis

Isolated primary cardiac angiosarcoma.
Evbayekha E, Rao AK, Leidenfrost J, Reiss CK
Curr Probl Cardiol 2024 May;49(5):102472. Epub 2024 Feb 17 doi: 10.1016/j.cpcardiol.2024.102472. PMID: 38369202
Primary Cardiac Angiosarcoma.
Guo S, Guo Q
Int Heart J 2021 Mar 30;62(2):458-462. Epub 2021 Mar 17 doi: 10.1536/ihj.20-581. PMID: 33731530
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
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
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

Therapy

Paclitaxel-induced dorsal hand-foot syndrome.
Quan EY, Engel C, Rush PS, Eikenberg JD
Dermatol Online J 2023 Jun 15;29(3) doi: 10.5070/D329361434. PMID: 37591274
Current Management of Angiosarcoma: Recent Advances and Lessons From the Past.
Florou V, Wilky BA
Curr Treat Options Oncol 2021 Jun 7;22(7):61. doi: 10.1007/s11864-021-00858-9. PMID: 34097172
Multidisciplinary Management of Angiosarcoma - A Review.
Sturm EC, Marasco IS, Katz SC
J Surg Res 2021 Jan;257:213-220. Epub 2020 Aug 25 doi: 10.1016/j.jss.2020.07.026. PMID: 32858322
Hepatic angiosarcoma.
Neshiwat LF, Friedland ML, Schorr-Lesnick B, Feldman S, Glucksman WJ, Russo RD Jr
Am J Med 1992 Aug;93(2):219-22. doi: 10.1016/0002-9343(92)90054-f. PMID: 1497020
Radiation-induced angiosarcoma.
Nanus DM, Kelsen D, Clark DG
Cancer 1987 Aug 15;60(4):777-9. doi: 10.1002/1097-0142(19870815)60:4<777::aid-cncr2820600412>3.0.co;2-t. PMID: 3297296

Prognosis

Hickam's dictum: Angiosarcoma-to-meningioma metastasis.
Roy CF, Zolotarov P, Roy SF, Razaghi F
Neuropathology 2019 Dec;39(6):447-451. Epub 2019 Oct 4 doi: 10.1111/neup.12591. PMID: 31584212
Primary renal angiosarcoma.
Gourley E, Digman G, Nicolas M, Kaushik D
BMJ Case Rep 2018 Jul 30;2018 doi: 10.1136/bcr-2017-222672. PMID: 30061122Free PMC Article
Angiosarcoma of the lung.
Grafino M, Alves P, de Almeida MM, Garrido P, Hasmucrai D, Teixeira E, Sotto-Mayor R
J Bras Pneumol 2016 Jan-Feb;42(1):68-70. doi: 10.1590/S1806-37562016000000193. PMID: 26982044Free PMC Article
Radiation-induced sarcoma.
Patel SR
Curr Treat Options Oncol 2000 Aug;1(3):258-61. doi: 10.1007/s11864-000-0037-6. PMID: 12057168
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

Clinical prediction guides

Primary epithelioid angiosarcoma of endometrium.
Sevim S, Gedik E, Ozakinci H, Kaygusuz G, Enneli D
Pol J Pathol 2023;74(3):219-222. doi: 10.5114/pjp.2023.132302. PMID: 37955542
Overview of malignant soft-tissue sarcomas of the limbs.
Renn A, Adejolu M, Messiou C, Bhaludin B, Strauss DC, Thway K, Moskovic E
Clin Radiol 2021 Dec;76(12):940.e1-940.e16. Epub 2021 Oct 1 doi: 10.1016/j.crad.2021.08.011. PMID: 34607656
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
Challenging epithelioid mesenchymal neoplasms: mimics and traps.
Cacciatore M, Dei Tos AP
Pathology 2014 Feb;46(2):126-34. doi: 10.1097/PAT.0000000000000063. PMID: 24384587
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

Recent systematic reviews

PD-L1 Expression in Cutaneous Angiosarcomas: A Systematic Review with Meta-Analysis.
Lobrano R, Paliogiannis P, Zinellu A, Palmieri G, Persico I, Mangoni AA, Cossu A
Curr Oncol 2023 May 17;30(5):5135-5144. doi: 10.3390/curroncol30050388. PMID: 37232846Free PMC Article
Primary angiosarcoma of the femoral artery in patient with kidney and liver polycystosis and multiple arterial aneurysms: report of the case and review of the literature.
Fabian O, Mokra D, Masopust J, Skorepa J, Kodetova DN, Zamecnik J
Cardiovasc Pathol 2019 Mar-Apr;39:8-11. Epub 2018 Nov 30 doi: 10.1016/j.carpath.2018.11.001. PMID: 30579128
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
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
Vinyl chloride exposure and cirrhosis: a systematic review and meta-analysis.
Frullanti E, La Vecchia C, Boffetta P, Zocchetti C
Dig Liver Dis 2012 Sep;44(9):775-9. Epub 2012 Mar 21 doi: 10.1016/j.dld.2012.02.007. PMID: 22440240

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

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

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • MeSH
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