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Localized Leiomyosarcoma

MedGen UID:
163507
•Concept ID:
C0855057
•
Neoplastic Process
Synonyms: Leiomyosarcoma Nonmetastatic; Non-Metastatic Leiomyosarcoma; Primary leiomyosarcoma
SNOMED CT: Primary leiomyosarcoma (423807009)

Definition

Leiomyosarcoma that is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocalized Leiomyosarcoma

Professional guidelines

PubMed

The biology and treatment of leiomyosarcomas.
Kerrison WGJ, Thway K, Jones RL, Huang PH
Crit Rev Oncol Hematol 2023 Apr;184:103955. Epub 2023 Mar 7 doi: 10.1016/j.critrevonc.2023.103955. PMID: 36893945
Retroperitoneal tumors: Review of diagnosis and management.
Sassa N
Int J Urol 2020 Dec;27(12):1058-1070. Epub 2020 Sep 10 doi: 10.1111/iju.14361. PMID: 32914475
Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment.
Llombart B, Serra-Guillén C, Requena C, Alsina M, Morgado-Carrasco D, Machado I, Sanmartín O
Actas Dermosifiliogr (Engl Ed) 2019 Jan-Feb;110(1):4-11. Epub 2018 Nov 5 doi: 10.1016/j.ad.2018.06.012. PMID: 30409380

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
Cutaneous Mesenchymal Sarcomas.
Walocko F, Christensen RE, Worley B, Alam M
Dermatol Clin 2023 Jan;41(1):133-140. Epub 2022 Oct 28 doi: 10.1016/j.det.2022.07.011. PMID: 36410974
Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
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
Uterine Leiomyosarcoma.
Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP
Oncol Res Treat 2018;41(11):680-686. Epub 2018 Oct 16 doi: 10.1159/000494299. PMID: 30321869

Diagnosis

Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Primary Renal Leiomyosarcoma.
Ding Y, Quan C
J Coll Physicians Surg Pak 2021 Jun;31(6):725-727. doi: 10.29271/jcpsp.2021.06.725. PMID: 34102790
Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment.
Llombart B, Serra-Guillén C, Requena C, Alsina M, Morgado-Carrasco D, Machado I, Sanmartín O
Actas Dermosifiliogr (Engl Ed) 2019 Jan-Feb;110(1):4-11. Epub 2018 Nov 5 doi: 10.1016/j.ad.2018.06.012. PMID: 30409380
Uterine Leiomyosarcoma.
Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP
Oncol Res Treat 2018;41(11):680-686. Epub 2018 Oct 16 doi: 10.1159/000494299. PMID: 30321869
Indications for morcellation in gynecologic surgery.
Parker WH
Curr Opin Obstet Gynecol 2018 Feb;30(1):75-80. doi: 10.1097/GCO.0000000000000427. PMID: 29251677

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
Preoperative Radiotherapy in Patients With Primary Retroperitoneal Sarcoma: EORTC-62092 Trial (STRASS) Versus Off-trial (STREXIT) Results.
Callegaro D, Raut CP, Ajayi T, Strauss D, Bonvalot S, Ng D, Stoeckle E, Fairweather M, Rutkowski P, van Houdt WJ, Gelderblom H, Sangalli C, Hayes A, Honoré C, Gladdy RA, Fau M, Haas R, Tzanis D, Miah AB, Chung P, Baldini EH, Marreaud S, Litiere S, Swallow CJ, Gronchi A
Ann Surg 2023 Jul 1;278(1):127-134. Epub 2022 Jul 14 doi: 10.1097/SLA.0000000000005492. PMID: 35833413
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
Hormone Replacement Therapy in Cancer Survivors - Review of the Literature.
Deli T, Orosz M, Jakab A
Pathol Oncol Res 2020 Jan;26(1):63-78. Epub 2019 Jan 8 doi: 10.1007/s12253-018-00569-x. PMID: 30617760Free PMC Article
Uterine Leiomyosarcoma.
Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP
Oncol Res Treat 2018;41(11):680-686. Epub 2018 Oct 16 doi: 10.1159/000494299. PMID: 30321869

Prognosis

The biology and treatment of leiomyosarcomas.
Kerrison WGJ, Thway K, Jones RL, Huang PH
Crit Rev Oncol Hematol 2023 Apr;184:103955. Epub 2023 Mar 7 doi: 10.1016/j.critrevonc.2023.103955. PMID: 36893945
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
Uterine Leiomyosarcoma.
Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP
Oncol Res Treat 2018;41(11):680-686. Epub 2018 Oct 16 doi: 10.1159/000494299. PMID: 30321869
Indications for morcellation in gynecologic surgery.
Parker WH
Curr Opin Obstet Gynecol 2018 Feb;30(1):75-80. doi: 10.1097/GCO.0000000000000427. PMID: 29251677
Cutaneous leiomyosarcoma.
Porter CJ, Januszkiewicz JS
Plast Reconstr Surg 2002 Mar;109(3):964-7. doi: 10.1097/00006534-200203000-00024. PMID: 11884817

Clinical prediction guides

Preoperative Radiotherapy in Patients With Primary Retroperitoneal Sarcoma: EORTC-62092 Trial (STRASS) Versus Off-trial (STREXIT) Results.
Callegaro D, Raut CP, Ajayi T, Strauss D, Bonvalot S, Ng D, Stoeckle E, Fairweather M, Rutkowski P, van Houdt WJ, Gelderblom H, Sangalli C, Hayes A, Honoré C, Gladdy RA, Fau M, Haas R, Tzanis D, Miah AB, Chung P, Baldini EH, Marreaud S, Litiere S, Swallow CJ, Gronchi A
Ann Surg 2023 Jul 1;278(1):127-134. Epub 2022 Jul 14 doi: 10.1097/SLA.0000000000005492. PMID: 35833413
Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Advances in MR Imaging of the Female Pelvis.
Sakala MD, Shampain KL, Wasnik AP
Magn Reson Imaging Clin N Am 2020 Aug;28(3):415-431. Epub 2020 Jun 6 doi: 10.1016/j.mric.2020.03.007. PMID: 32624159
Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment.
Llombart B, Serra-Guillén C, Requena C, Alsina M, Morgado-Carrasco D, Machado I, Sanmartín O
Actas Dermosifiliogr (Engl Ed) 2019 Jan-Feb;110(1):4-11. Epub 2018 Nov 5 doi: 10.1016/j.ad.2018.06.012. PMID: 30409380
Uterine Leiomyosarcoma.
Juhasz-Böss I, Gabriel L, Bohle RM, Horn LC, Solomayer EF, Breitbach GP
Oncol Res Treat 2018;41(11):680-686. Epub 2018 Oct 16 doi: 10.1159/000494299. PMID: 30321869

Recent systematic reviews

Evaluation of the efficacy of systemic therapy for advanced uterine leiomyosarcoma: A systematic review, meta-analysis, and meta-regression analysis.
Ijaz I, Shahzad MN, Hosseinifard H, Liu S, Sefidan MO, Kahloon LE, Imani S, Hua Z, Zhang YQ
Cancer Med 2023 Jul;12(13):13894-13911. Epub 2023 Apr 20 doi: 10.1002/cam4.5930. PMID: 37081717Free PMC Article
Leiomyosarcoma in the extremities and trunk wall: systematic review and meta-analysis of the oncological outcomes.
Kannan S, Chong HH, Chew B, Ferguson JD, Galloway E, McCulloch T, Rankin KS, Ashford RU
World J Surg Oncol 2022 Apr 18;20(1):124. doi: 10.1186/s12957-022-02584-4. PMID: 35436892Free PMC Article
Isolated Limb Perfusion and Infusion for Extremity Soft Tissue Sarcoma: A Contemporary Systematic Review and Meta-Analysis.
Neuwirth MG, Song Y, Sinnamon AJ, Fraker DL, Zager JS, Karakousis GC
Ann Surg Oncol 2017 Dec;24(13):3803-3810. Epub 2017 Oct 11 doi: 10.1245/s10434-017-6109-7. PMID: 29022281
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
Systematic chemotherapy for inoperable, locally advanced, recurrent, or metastatic uterine leiomyosarcoma: a systematic review.
Gupta AA, Yao X, Verma S, Mackay H, Hopkins L; Sarcoma Disease Site Group and the Gynecology Cancer Disease Site Group
Clin Oncol (R Coll Radiol) 2013 Jun;25(6):346-55. Epub 2013 Jan 5 doi: 10.1016/j.clon.2012.11.008. PMID: 23295078

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