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Leiomyosarcoma NCI Grade 3

MedGen UID:
233612
•Concept ID:
C1334384
•
Neoplastic Process
Synonym: Leiomyosarcoma NCI Grade III

Definition

A leiomyosarcoma with more than 15% necrosis. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Amplitude-modulated electromagnetic fields for the treatment of cancer: discovery of tumor-specific frequencies and assessment of a novel therapeutic approach.
Barbault A, Costa FP, Bottger B, Munden RF, Bomholt F, Kuster N, Pasche B
J Exp Clin Cancer Res 2009 Apr 14;28(1):51. doi: 10.1186/1756-9966-28-51. PMID: 19366446Free PMC Article

Recent clinical studies

Etiology

Phase 3, randomized, open-label study of pembrolizumab plus lenvatinib versus chemotherapy for first-line treatment of advanced or recurrent endometrial cancer: ENGOT-en9/LEAP-001.
Marth C, Tarnawski R, Tyulyandina A, Pignata S, Gilbert L, Kaen D, Rubio MJ, Frentzas S, Beiner M, Magallanes-Maciel M, Farrelly L, Choi CH, Berger R, Lee C, Vulsteke C, Hasegawa K, Braicu EI, Wu X, McKenzie J, Lee JJ, Makker V
Int J Gynecol Cancer 2022 Jan;32(1):93-100. Epub 2021 Nov 19 doi: 10.1136/ijgc-2021-003017. PMID: 34799418Free PMC Article
Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free PMC Article
Effect of Doxorubicin Plus Olaratumab vs Doxorubicin Plus Placebo on Survival in Patients With Advanced Soft Tissue Sarcomas: The ANNOUNCE Randomized Clinical Trial.
Tap WD, Wagner AJ, Schöffski P, Martin-Broto J, Krarup-Hansen A, Ganjoo KN, Yen CC, Abdul Razak AR, Spira A, Kawai A, Le Cesne A, Van Tine BA, Naito Y, Park SH, Fedenko A, Pápai Z, Soldatenkova V, Shahir A, Mo G, Wright J, Jones RL; ANNOUNCE Investigators
JAMA 2020 Apr 7;323(13):1266-1276. doi: 10.1001/jama.2020.1707. PMID: 32259228Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial.
Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR
J Clin Oncol 2016 Mar 10;34(8):786-93. Epub 2015 Sep 14 doi: 10.1200/JCO.2015.62.4734. PMID: 26371143Free PMC Article

Diagnosis

Grade of Primary Cutaneous Leiomyosarcoma Dictates Risk for Metastatic Spread and Disease-Specific Mortality.
Carr MJ, Sun J, Adams WA, Dugan MM, Naqvi SMH, Kim Y, Messina JL, Gonzalez RJ, Zager JS, Mullinax JE
Cancer Control 2023 Jan-Dec;30:10732748231206957. doi: 10.1177/10732748231206957. PMID: 37876208Free PMC Article
Molecular-Based Immunohistochemical Algorithm for Uterine Leiomyosarcoma Diagnosis.
Momeni-Boroujeni A, Yousefi E, Balakrishnan R, Riviere S, Kertowidjojo E, Hensley ML, Ladanyi M, Ellenson LH, Chiang S
Mod Pathol 2023 Apr;36(4):100084. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100084. PMID: 36788080Free PMC Article
Phase 3, randomized, open-label study of pembrolizumab plus lenvatinib versus chemotherapy for first-line treatment of advanced or recurrent endometrial cancer: ENGOT-en9/LEAP-001.
Marth C, Tarnawski R, Tyulyandina A, Pignata S, Gilbert L, Kaen D, Rubio MJ, Frentzas S, Beiner M, Magallanes-Maciel M, Farrelly L, Choi CH, Berger R, Lee C, Vulsteke C, Hasegawa K, Braicu EI, Wu X, McKenzie J, Lee JJ, Makker V
Int J Gynecol Cancer 2022 Jan;32(1):93-100. Epub 2021 Nov 19 doi: 10.1136/ijgc-2021-003017. PMID: 34799418Free PMC Article
Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free 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

Phase II Study of Olaparib and Temozolomide for Advanced Uterine Leiomyosarcoma (NCI Protocol 10250).
Ingham M, Allred JB, Chen L, Das B, Kochupurakkal B, Gano K, George S, Attia S, Burgess MA, Seetharam M, Boikos SA, Bui N, Chen JL, Close JL, Cote GM, Thaker PH, Ivy SP, Bose S, D'Andrea A, Marino-Enriquez A, Shapiro GI, Schwartz GK
J Clin Oncol 2023 Sep 1;41(25):4154-4163. Epub 2023 Jul 19 doi: 10.1200/JCO.23.00402. PMID: 37467452Free PMC Article
Effect of Capivasertib in Patients With an AKT1 E17K-Mutated Tumor: NCI-MATCH Subprotocol EAY131-Y Nonrandomized Trial.
Kalinsky K, Hong F, McCourt CK, Sachdev JC, Mitchell EP, Zwiebel JA, Doyle LA, McShane LM, Li S, Gray RJ, Rubinstein LV, Patton D, Williams PM, Hamilton SR, Conley BA, O'Dwyer PJ, Harris LN, Arteaga CL, Chen AP, Flaherty KT
JAMA Oncol 2021 Feb 1;7(2):271-278. doi: 10.1001/jamaoncol.2020.6741. PMID: 33377972Free PMC Article
Effect of Doxorubicin Plus Olaratumab vs Doxorubicin Plus Placebo on Survival in Patients With Advanced Soft Tissue Sarcomas: The ANNOUNCE Randomized Clinical Trial.
Tap WD, Wagner AJ, Schöffski P, Martin-Broto J, Krarup-Hansen A, Ganjoo KN, Yen CC, Abdul Razak AR, Spira A, Kawai A, Le Cesne A, Van Tine BA, Naito Y, Park SH, Fedenko A, Pápai Z, Soldatenkova V, Shahir A, Mo G, Wright J, Jones RL; ANNOUNCE Investigators
JAMA 2020 Apr 7;323(13):1266-1276. doi: 10.1001/jama.2020.1707. PMID: 32259228Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial.
Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR
J Clin Oncol 2016 Mar 10;34(8):786-93. Epub 2015 Sep 14 doi: 10.1200/JCO.2015.62.4734. PMID: 26371143Free PMC Article

Prognosis

Sarcomas With RAD51B Fusions Are Associated With a Heterogeneous Phenotype.
Chang HY, Dermawan J, Sharma A, Dickson B, Turashvili G, Torrence D, Nucci M, Chiang S, Oliva E, Kirchner M, Stenzinger A, Mechtersheimer G, Antonescu C
Mod Pathol 2024 Feb;37(2):100402. Epub 2023 Dec 21 doi: 10.1016/j.modpat.2023.100402. PMID: 38141829Free PMC Article
Grade of Primary Cutaneous Leiomyosarcoma Dictates Risk for Metastatic Spread and Disease-Specific Mortality.
Carr MJ, Sun J, Adams WA, Dugan MM, Naqvi SMH, Kim Y, Messina JL, Gonzalez RJ, Zager JS, Mullinax JE
Cancer Control 2023 Jan-Dec;30:10732748231206957. doi: 10.1177/10732748231206957. PMID: 37876208Free PMC Article
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.
Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S
Lancet Oncol 2017 Nov;18(11):1493-1501. Epub 2017 Oct 4 doi: 10.1016/S1470-2045(17)30624-1. PMID: 28988646Free PMC Article
Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial.
Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR
J Clin Oncol 2016 Mar 10;34(8):786-93. Epub 2015 Sep 14 doi: 10.1200/JCO.2015.62.4734. PMID: 26371143Free 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: 36971773Free PMC Article
Effect of Doxorubicin Plus Olaratumab vs Doxorubicin Plus Placebo on Survival in Patients With Advanced Soft Tissue Sarcomas: The ANNOUNCE Randomized Clinical Trial.
Tap WD, Wagner AJ, Schöffski P, Martin-Broto J, Krarup-Hansen A, Ganjoo KN, Yen CC, Abdul Razak AR, Spira A, Kawai A, Le Cesne A, Van Tine BA, Naito Y, Park SH, Fedenko A, Pápai Z, Soldatenkova V, Shahir A, Mo G, Wright J, Jones RL; ANNOUNCE Investigators
JAMA 2020 Apr 7;323(13):1266-1276. doi: 10.1001/jama.2020.1707. PMID: 32259228Free PMC Article
Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review.
Cheng H, Miura JT, Lalehzari M, Rajeev R, Donahue AE, Bedi M, Gamblin TC, Turaga KK, Johnston FM
J Surg Oncol 2016 May;113(6):628-34. Epub 2016 Mar 16 doi: 10.1002/jso.24221. PMID: 26990903
Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial.
Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR
J Clin Oncol 2016 Mar 10;34(8):786-93. Epub 2015 Sep 14 doi: 10.1200/JCO.2015.62.4734. PMID: 26371143Free 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

Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review.
Cheng H, Miura JT, Lalehzari M, Rajeev R, Donahue AE, Bedi M, Gamblin TC, Turaga KK, Johnston FM
J Surg Oncol 2016 May;113(6):628-34. Epub 2016 Mar 16 doi: 10.1002/jso.24221. PMID: 26990903

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