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Unresectable Soft Tissue Leiomyosarcoma

MedGen UID:
1713501
•Concept ID:
C5239080
•
Neoplastic Process

Definition

A soft tissue leiomyosarcoma that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable Soft Tissue Leiomyosarcoma

Professional guidelines

PubMed

Surgical Management of Retroperitoneal Sarcoma.
Dominguez DA, Sampath S, Agulnik M, Liang Y, Nguyen B, Trisal V, Melstrom LG, Lewis AG, Paz IB, Roberts RF, Tseng WW
Curr Oncol 2023 Apr 29;30(5):4618-4631. doi: 10.3390/curroncol30050349. PMID: 37232807Free PMC Article
Efficacy of trabectedin for the treatment of liposarcoma.
Zijoo R, von Mehren M
Expert Opin Pharmacother 2016 Oct;17(14):1953-62. Epub 2016 Sep 15 doi: 10.1080/14656566.2016.1229304. PMID: 27615729
Management of gastrointestinal stromal tumors: from diagnosis to treatment.
Bucher P, Villiger P, Egger JF, Buhler LH, Morel P
Swiss Med Wkly 2004 Mar 20;134(11-12):145-53. doi: 10.4414/smw.2004.10530. PMID: 15106018

Recent clinical studies

Etiology

Doxorubicin-Trabectedin with Trabectedin Maintenance in Leiomyosarcoma.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Le Cesne A, Balleyguier C, Archambaud B, Duffaud F; French Sarcoma Group
N Engl J Med 2024 Sep 5;391(9):789-799. doi: 10.1056/NEJMoa2403394. PMID: 39231341
Surgical Management of Retroperitoneal Sarcoma.
Dominguez DA, Sampath S, Agulnik M, Liang Y, Nguyen B, Trisal V, Melstrom LG, Lewis AG, Paz IB, Roberts RF, Tseng WW
Curr Oncol 2023 Apr 29;30(5):4618-4631. doi: 10.3390/curroncol30050349. PMID: 37232807Free PMC Article
Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
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

Diagnosis

Pazopanib-induced fatal heart failure in a patient with unresectable soft tissue sarcoma and review of literature.
Karaağaç M, Eryılmaz MK
J Oncol Pharm Pract 2020 Apr;26(3):768-774. Epub 2019 Sep 23 doi: 10.1177/1078155219875797. PMID: 31547750
Prolonged disease stability with trabectedin in a heavily pretreated elderly patient with metastatic leiomyosarcoma of the thigh and renal failure: a case report and review of the literature.
Galizia D, Palesandro E, Nuzzo AM, Pignochino Y, Aliberti S, Aglietta M, Grignani G
Oncol Res 2013;20(10):483-90. doi: 10.3727/096504013x13685487925130. PMID: 24308159
Docetaxel and gemcitabine combination in 133 advanced soft-tissue sarcomas: a retrospective analysis.
Bay JO, Ray-Coquard I, Fayette J, Leyvraz S, Cherix S, Piperno-Neumann S, Chevreau C, Isambert N, Brain E, Emile G, Le Cesne A, Cioffi A, Kwiatkowski F, Coindre JM, Bui NB, Peyrade F, Penel N, Blay JY; Groupe Sarcome Français
Int J Cancer 2006 Aug 1;119(3):706-11. doi: 10.1002/ijc.21867. PMID: 16496406
Temozolomide in uterine leiomyosarcomas.
Anderson S, Aghajanian C
Gynecol Oncol 2005 Jul;98(1):99-103. doi: 10.1016/j.ygyno.2005.03.018. PMID: 15916799
Management of gastrointestinal stromal tumors: from diagnosis to treatment.
Bucher P, Villiger P, Egger JF, Buhler LH, Morel P
Swiss Med Wkly 2004 Mar 20;134(11-12):145-53. doi: 10.4414/smw.2004.10530. PMID: 15106018

Therapy

Doxorubicin-Trabectedin with Trabectedin Maintenance in Leiomyosarcoma.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Le Cesne A, Balleyguier C, Archambaud B, Duffaud F; French Sarcoma Group
N Engl J Med 2024 Sep 5;391(9):789-799. doi: 10.1056/NEJMoa2403394. PMID: 39231341
Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
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 of trabectedin for the treatment of liposarcoma.
Zijoo R, von Mehren M
Expert Opin Pharmacother 2016 Oct;17(14):1953-62. Epub 2016 Sep 15 doi: 10.1080/14656566.2016.1229304. PMID: 27615729

Prognosis

Doxorubicin alone versus doxorubicin with trabectedin followed by trabectedin alone as first-line therapy for metastatic or unresectable leiomyosarcoma (LMS-04): a randomised, multicentre, open-label phase 3 trial.
Pautier P, Italiano A, Piperno-Neumann S, Chevreau C, Penel N, Firmin N, Boudou-Rouquette P, Bertucci F, Balleyguier C, Lebrun-Ly V, Ray-Coquard I, Kalbacher E, Bardet A, Bompas E, Collard O, Isambert N, Guillemet C, Rios M, Archambaud B, Duffaud F; French Sarcoma Group
Lancet Oncol 2022 Aug;23(8):1044-1054. Epub 2022 Jul 11 doi: 10.1016/S1470-2045(22)00380-1. PMID: 35835135
Efficacy and safety of trabectedin for patients with unresectable and relapsed soft-tissue sarcoma in Japan: A Japanese Musculoskeletal Oncology Group study.
Kobayashi H, Iwata S, Wakamatsu T, Hayakawa K, Yonemoto T, Wasa J, Oka H, Ueda T, Tanaka S
Cancer 2020 Mar 15;126(6):1253-1263. Epub 2019 Dec 11 doi: 10.1002/cncr.32661. PMID: 31825533
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
Management of gastrointestinal stromal tumors: from diagnosis to treatment.
Bucher P, Villiger P, Egger JF, Buhler LH, Morel P
Swiss Med Wkly 2004 Mar 20;134(11-12):145-53. doi: 10.4414/smw.2004.10530. PMID: 15106018
The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.
Pirayesh A, Chee Y, Helliwell TR, Hershman MJ, Leinster SJ, Fordham MV, Poston GJ
Eur J Surg Oncol 2001 Aug;27(5):491-7. doi: 10.1053/ejso.2001.1146. PMID: 11504522

Clinical prediction guides

Mesna, Doxorubicin, Ifosfamide and Dacarbazine (MAID) Combination Chemotherapy for Retroperitoneal Sarcoma: A Single-center Experience.
Ishiyama Y, Inakawa T, Yoshino M, Nishimura K, Horiuchi T, Ishihara H, Toki D, Kondo T
Anticancer Res 2024 Jul;44(7):3213-3220. doi: 10.21873/anticanres.17137. PMID: 38925814
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
First-Line Aldoxorubicin vs Doxorubicin in Metastatic or Locally Advanced Unresectable Soft-Tissue Sarcoma: A Phase 2b Randomized Clinical Trial.
Chawla SP, Papai Z, Mukhametshina G, Sankhala K, Vasylyev L, Fedenko A, Khamly K, Ganjoo K, Nagarkar R, Wieland S, Levitt DJ
JAMA Oncol 2015 Dec;1(9):1272-80. doi: 10.1001/jamaoncol.2015.3101. PMID: 26378637
Docetaxel and gemcitabine combination in 133 advanced soft-tissue sarcomas: a retrospective analysis.
Bay JO, Ray-Coquard I, Fayette J, Leyvraz S, Cherix S, Piperno-Neumann S, Chevreau C, Isambert N, Brain E, Emile G, Le Cesne A, Cioffi A, Kwiatkowski F, Coindre JM, Bui NB, Peyrade F, Penel N, Blay JY; Groupe Sarcome Français
Int J Cancer 2006 Aug 1;119(3):706-11. doi: 10.1002/ijc.21867. PMID: 16496406
Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
Cecchetto G, Alaggio R, Dall'Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M
Cancer 2005 Nov 1;104(9):2006-12. doi: 10.1002/cncr.21412. PMID: 16161038

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