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Angiosarcoma NCI Grade 3

MedGen UID:
272314
•Concept ID:
C1332299
•
Neoplastic Process
Synonym: Angiosarcoma NCI Grade III

Definition

An angiosarcoma with more than 15% necrosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAngiosarcoma NCI Grade 3

Professional guidelines

PubMed

Treatment and outcome of 82 patients with angiosarcoma.
Abraham JA, Hornicek FJ, Kaufman AM, Harmon DC, Springfield DS, Raskin KA, Mankin HJ, Kirsch DG, Rosenberg AE, Nielsen GP, Desphpande V, Suit HD, DeLaney TF, Yoon SS
Ann Surg Oncol 2007 Jun;14(6):1953-67. Epub 2007 Mar 14 doi: 10.1245/s10434-006-9335-y. PMID: 17356953

Recent clinical studies

Etiology

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: 34380663Free PMC Article
An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma.
Agulnik M, Schulte B, Robinson S, Hirbe AC, Kozak K, Chawla SP, Attia S, Rademaker A, Zhang H, Abbinanti S, Cehic R, Monga V, Milhem M, Okuno S, Van Tine BA
Eur J Cancer 2021 Sep;154:201-208. Epub 2021 Jul 17 doi: 10.1016/j.ejca.2021.06.027. PMID: 34284255
Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets.
Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Primary and secondary breast angiosarcoma: single center report and a meta-analysis.
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
Adult urological sarcoma.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, Brennan MF
J Urol 1992 Apr;147(4):1032-6; discussion 1036-7. doi: 10.1016/s0022-5347(17)37456-6. PMID: 1552580

Diagnosis

Histopathologic Grading Is of Prognostic Significance in Primary Angiosarcoma of Breast: Proposal of a Simplified 2-tier Grading System.
Kuba MG, Dermawan JK, Xu B, Singer S, Plitas G, Tap WD, D'Angelo SP, Rosenbaum E, Brogi E, Antonescu CR
Am J Surg Pathol 2023 Mar 1;47(3):307-317. Epub 2022 Nov 15 doi: 10.1097/PAS.0000000000001998. PMID: 36376999Free PMC Article
Hepatic small vessel neoplasm, a rare infiltrative vascular neoplasm of uncertain malignant potential.
Gill RM, Buelow B, Mather C, Joseph NM, Alves V, Brunt EM, Liu TC, Makhlouf H, Marginean C, Nalbantoglu I, Sempoux C, Snover DC, Thung SN, Yeh MM, Ferrell LD
Hum Pathol 2016 Aug;54:143-51. Epub 2016 Apr 14 doi: 10.1016/j.humpath.2016.03.018. PMID: 27090685Free PMC Article
Treatment and outcome of 82 patients with angiosarcoma.
Abraham JA, Hornicek FJ, Kaufman AM, Harmon DC, Springfield DS, Raskin KA, Mankin HJ, Kirsch DG, Rosenberg AE, Nielsen GP, Desphpande V, Suit HD, DeLaney TF, Yoon SS
Ann Surg Oncol 2007 Jun;14(6):1953-67. Epub 2007 Mar 14 doi: 10.1245/s10434-006-9335-y. PMID: 17356953
Cutaneous angiosarcoma of the scalp: a multidisciplinary approach.
Pawlik TM, Paulino AF, McGinn CJ, Baker LH, Cohen DS, Morris JS, Rees R, Sondak VK
Cancer 2003 Oct 15;98(8):1716-26. doi: 10.1002/cncr.11667. PMID: 14534889
Adult urological sarcoma.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, Brennan MF
J Urol 1992 Apr;147(4):1032-6; discussion 1036-7. doi: 10.1016/s0022-5347(17)37456-6. PMID: 1552580

Therapy

A Phase II Study of Epacadostat and Pembrolizumab in Patients with Advanced Sarcoma.
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Single-agent gemcitabine in patients with advanced, pre-treated angiosarcoma: A multicenter, retrospective study.
Watson S, Verret B, Ropert S, Adam J, Bahleda R, Briand S, Cavalcanti A, Chamseddine AN, Court C, Fadel E, Faron M, Haddag-Miliani L, Henon C, Pechoux CL, Levy A, Mercier O, Ngo C, Honoré C, Cesne AL, Mir O
Cancer Med 2023 Feb;12(3):3160-3166. Epub 2022 Aug 15 doi: 10.1002/cam4.5147. PMID: 35971325Free PMC Article
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: 34380663Free PMC Article
An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma.
Agulnik M, Schulte B, Robinson S, Hirbe AC, Kozak K, Chawla SP, Attia S, Rademaker A, Zhang H, Abbinanti S, Cehic R, Monga V, Milhem M, Okuno S, Van Tine BA
Eur J Cancer 2021 Sep;154:201-208. Epub 2021 Jul 17 doi: 10.1016/j.ejca.2021.06.027. PMID: 34284255
Phase II study of MLN8237 (Alisertib) in advanced/metastatic sarcoma.
Dickson MA, Mahoney MR, Tap WD, D'Angelo SP, Keohan ML, Van Tine BA, Agulnik M, Horvath LE, Nair JS, Schwartz GK
Ann Oncol 2016 Oct;27(10):1855-60. Epub 2016 Aug 8 doi: 10.1093/annonc/mdw281. PMID: 27502708Free PMC Article

Prognosis

Histopathologic Grading Is of Prognostic Significance in Primary Angiosarcoma of Breast: Proposal of a Simplified 2-tier Grading System.
Kuba MG, Dermawan JK, Xu B, Singer S, Plitas G, Tap WD, D'Angelo SP, Rosenbaum E, Brogi E, Antonescu CR
Am J Surg Pathol 2023 Mar 1;47(3):307-317. Epub 2022 Nov 15 doi: 10.1097/PAS.0000000000001998. PMID: 36376999Free PMC Article
Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets.
Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Primary and secondary breast angiosarcoma: single center report and a meta-analysis.
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
Treatment and outcome of 82 patients with angiosarcoma.
Abraham JA, Hornicek FJ, Kaufman AM, Harmon DC, Springfield DS, Raskin KA, Mankin HJ, Kirsch DG, Rosenberg AE, Nielsen GP, Desphpande V, Suit HD, DeLaney TF, Yoon SS
Ann Surg Oncol 2007 Jun;14(6):1953-67. Epub 2007 Mar 14 doi: 10.1245/s10434-006-9335-y. PMID: 17356953
Adult urological sarcoma.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, Brennan MF
J Urol 1992 Apr;147(4):1032-6; discussion 1036-7. doi: 10.1016/s0022-5347(17)37456-6. PMID: 1552580

Clinical prediction guides

A Phase II Study of Epacadostat and Pembrolizumab in Patients with Advanced Sarcoma.
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Histopathologic Grading Is of Prognostic Significance in Primary Angiosarcoma of Breast: Proposal of a Simplified 2-tier Grading System.
Kuba MG, Dermawan JK, Xu B, Singer S, Plitas G, Tap WD, D'Angelo SP, Rosenbaum E, Brogi E, Antonescu CR
Am J Surg Pathol 2023 Mar 1;47(3):307-317. Epub 2022 Nov 15 doi: 10.1097/PAS.0000000000001998. PMID: 36376999Free PMC Article
Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets.
Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Primary and secondary breast angiosarcoma: single center report and a meta-analysis.
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
Adult urological sarcoma.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, Brennan MF
J Urol 1992 Apr;147(4):1032-6; discussion 1036-7. doi: 10.1016/s0022-5347(17)37456-6. PMID: 1552580

Recent systematic reviews

Primary and secondary breast angiosarcoma: single center report and a meta-analysis.
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

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