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Unresectable Leiomyosarcoma

MedGen UID:
1385549
•Concept ID:
C4331475
•
Neoplastic Process

Definition

A leiomyosarcoma which is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable 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
Systemic treatment options for radiation-associated sarcomas.
Dickson MA
Curr Treat Options Oncol 2014 Sep;15(3):476-81. doi: 10.1007/s11864-014-0299-z. PMID: 24968875

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
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

Diagnosis

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

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
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
Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial.
Hensley ML, Maki R, Venkatraman E, Geller G, Lovegren M, Aghajanian C, Sabbatini P, Tong W, Barakat R, Spriggs DR
J Clin Oncol 2002 Jun 15;20(12):2824-31. doi: 10.1200/JCO.2002.11.050. PMID: 12065559
Leiomyosarcoma of the kidney: report of a patient with favorable response to doxorubicin and cisplatin suspended in a lipid contrast medium and cyclophosphamide.
Taniguchi H, Takahashi T, Fujita Y, Sakita M, Ogita S, Sawai K, Yamaguchi T, Yokota T, Nakagawa N, Shimotsuma M
Med Pediatr Oncol 1987;15(5):285-90. doi: 10.1002/mpo.2950150513. PMID: 3657716

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

Clinical prediction guides

Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial.
Hensley ML, Maki R, Venkatraman E, Geller G, Lovegren M, Aghajanian C, Sabbatini P, Tong W, Barakat R, Spriggs DR
J Clin Oncol 2002 Jun 15;20(12):2824-31. doi: 10.1200/JCO.2002.11.050. PMID: 12065559

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