U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
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
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

Diagnosis

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
Surgical resection for recurrent retroperitoneal leiomyosarcoma and liposarcoma.
Nathenson MJ, Barysauskas CM, Nathenson RA, Regine WF, Hanna N, Sausville E
World J Surg Oncol 2018 Oct 11;16(1):203. doi: 10.1186/s12957-018-1505-4. PMID: 30309356Free 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 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
A Phase II Trial of Sorafenib and Dacarbazine for Leiomyosarcoma, Synovial Sarcoma, and Malignant Peripheral Nerve Sheath Tumors.
D'Adamo DR, Dickson MA, Keohan ML, Carvajal RD, Hensley ML, Hirst CM, Ezeoke MO, Ahn L, Qin LX, Antonescu CR, Lefkowitz RA, Maki RG, Schwartz GK, Tap WD
Oncologist 2019 Jun;24(6):857-863. Epub 2018 Aug 20 doi: 10.1634/theoncologist.2018-0160. PMID: 30126857Free 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
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

Prognosis

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
Surgical resection for recurrent retroperitoneal leiomyosarcoma and liposarcoma.
Nathenson MJ, Barysauskas CM, Nathenson RA, Regine WF, Hanna N, Sausville E
World J Surg Oncol 2018 Oct 11;16(1):203. doi: 10.1186/s12957-018-1505-4. PMID: 30309356Free 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

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
The enigma of myxofibrosarcoma of the extremity.
Mutter RW, Singer S, Zhang Z, Brennan MF, Alektiar KM
Cancer 2012 Jan 15;118(2):518-27. Epub 2011 Jun 29 doi: 10.1002/cncr.26296. PMID: 21717447Free PMC Article
Clinical presentation and outcome of high-grade urinary bladder leiomyosarcoma in adults.
Rosser CJ, Slaton JW, Izawa JI, Levy LB, Dinney CP
Urology 2003 Jun;61(6):1151-5. doi: 10.1016/s0090-4295(03)00021-9. PMID: 12809885
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

Supplemental Content

Table of contents

    Practice guidelines

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

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...