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Angiosarcoma NCI Grade 2

MedGen UID:
233987
•Concept ID:
C1332298
•
Neoplastic Process
Synonym: Angiosarcoma NCI Grade II

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAngiosarcoma NCI Grade 2

Professional guidelines

PubMed

Treatment Approaches and Outcomes for Primary Mediastinal Sarcoma: Analysis of 976 Patients.
Engelhardt KE, DeCamp MM, Yang AD, Bilimoria KY, Odell DD
Ann Thorac Surg 2018 Aug;106(2):333-339. Epub 2018 May 2 doi: 10.1016/j.athoracsur.2018.03.068. PMID: 29729268Free PMC Article

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
Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.
Palmerini E, Maki RG, Staals EL, Alberghini M, Antonescu CR, Ferrari C, Ruggieri P, Mavrogenis A, Bertoni F, Cesari M, Paioli A, Marchesi E, Picci P, Ferrari S
Am J Clin Oncol 2014 Dec;37(6):528-34. doi: 10.1097/COC.0b013e31827defa1. PMID: 23466575Free PMC Article
Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas?
Gladdy RA, Qin LX, Moraco N, Edgar MA, Antonescu CR, Alektiar KM, Brennan MF, Singer S
J Clin Oncol 2010 Apr 20;28(12):2064-9. Epub 2010 Mar 22 doi: 10.1200/JCO.2009.25.1728. PMID: 20308666Free 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

Mesenchymal Tumors Involving the Pancreas: A Clinicopathologic Analysis and Review of the Literature.
Askan G, Basturk O
Turk Patoloji Derg 2022;38(1):46-53. doi: 10.5146/tjpath.2022.01567. PMID: 35001360Free PMC Article
Treatment Approaches and Outcomes for Primary Mediastinal Sarcoma: Analysis of 976 Patients.
Engelhardt KE, DeCamp MM, Yang AD, Bilimoria KY, Odell DD
Ann Thorac Surg 2018 Aug;106(2):333-339. Epub 2018 May 2 doi: 10.1016/j.athoracsur.2018.03.068. PMID: 29729268Free 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
Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.
Palmerini E, Maki RG, Staals EL, Alberghini M, Antonescu CR, Ferrari C, Ruggieri P, Mavrogenis A, Bertoni F, Cesari M, Paioli A, Marchesi E, Picci P, Ferrari S
Am J Clin Oncol 2014 Dec;37(6):528-34. doi: 10.1097/COC.0b013e31827defa1. PMID: 23466575Free 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

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: 36971773
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
Alliance A091103 a phase II study of the angiopoietin 1 and 2 peptibody trebananib for the treatment of angiosarcoma.
D' Angelo SP, Mahoney MR, Van Tine BA, Adkins DR, Perdekamp MT, Condy MM, Luke JJ, Hartley EW, Antonescu CR, Tap WD, Schwartz GK
Cancer Chemother Pharmacol 2015 Mar;75(3):629-38. Epub 2015 Feb 12 doi: 10.1007/s00280-015-2689-8. PMID: 25672915Free PMC Article

Prognosis

Treatment Approaches and Outcomes for Primary Mediastinal Sarcoma: Analysis of 976 Patients.
Engelhardt KE, DeCamp MM, Yang AD, Bilimoria KY, Odell DD
Ann Thorac Surg 2018 Aug;106(2):333-339. Epub 2018 May 2 doi: 10.1016/j.athoracsur.2018.03.068. PMID: 29729268Free 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
Alliance A091103 a phase II study of the angiopoietin 1 and 2 peptibody trebananib for the treatment of angiosarcoma.
D' Angelo SP, Mahoney MR, Van Tine BA, Adkins DR, Perdekamp MT, Condy MM, Luke JJ, Hartley EW, Antonescu CR, Tap WD, Schwartz GK
Cancer Chemother Pharmacol 2015 Mar;75(3):629-38. Epub 2015 Feb 12 doi: 10.1007/s00280-015-2689-8. PMID: 25672915Free PMC Article
Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas?
Gladdy RA, Qin LX, Moraco N, Edgar MA, Antonescu CR, Alektiar KM, Brennan MF, Singer S
J Clin Oncol 2010 Apr 20;28(12):2064-9. Epub 2010 Mar 22 doi: 10.1200/JCO.2009.25.1728. PMID: 20308666Free 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

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: 36971773
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
Alliance A091103 a phase II study of the angiopoietin 1 and 2 peptibody trebananib for the treatment of angiosarcoma.
D' Angelo SP, Mahoney MR, Van Tine BA, Adkins DR, Perdekamp MT, Condy MM, Luke JJ, Hartley EW, Antonescu CR, Tap WD, Schwartz GK
Cancer Chemother Pharmacol 2015 Mar;75(3):629-38. Epub 2015 Feb 12 doi: 10.1007/s00280-015-2689-8. PMID: 25672915Free PMC Article
Dual VEGF/VEGFR inhibition in advanced solid malignancies: clinical effects and pharmacodynamic biomarkers.
Mittal K, Koon H, Elson P, Triozzi P, Dowlati A, Chen H, Borden EC, Rini BI
Cancer Biol Ther 2014 Aug;15(8):975-81. Epub 2014 May 19 doi: 10.4161/cbt.29187. PMID: 24842548Free 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

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