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Metastatic Soft Tissue Leiomyosarcoma

MedGen UID:
1714888
•Concept ID:
C5239078
•
Neoplastic Process

Definition

A soft tissue leiomyosarcoma that has spread from the original site of growth to another anatomic site. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Leiomyosarcoma: Current Clinical Management and Future Horizons.
Devaud N, Vornicova O, Abdul Razak AR, Khalili K, Demicco EG, Mitric C, Bernardini MQ, Gladdy RA
Surg Oncol Clin N Am 2022 Jul;31(3):527-546. doi: 10.1016/j.soc.2022.03.011. PMID: 35715148
What Clinical Trials Are Needed for Treatment of Leiomyosarcoma?
Kasper B, D'Ambrosio L, Davis EJ, Ingham M, Broto JM, Trent JC, van Houdt WJ, Van Tine BA
Curr Treat Options Oncol 2022 Mar;23(3):439-449. Epub 2022 Mar 11 doi: 10.1007/s11864-021-00928-y. PMID: 35275323Free PMC Article
Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: A propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.
D'Ambrosio L, Touati N, Blay JY, Grignani G, Flippot R, Czarnecka AM, Piperno-Neumann S, Martin-Broto J, Sanfilippo R, Katz D, Duffaud F, Vincenzi B, Stark DP, Mazzeo F, Tuchscherer A, Chevreau C, Sherriff J, Estival A, Litière S, Sents W, Ray-Coquard I, Tolomeo F, Le Cesne A, Rutkowski P, Stacchiotti S, Kasper B, Gelderblom H, Gronchi A; European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Cancer 2020 Jun 1;126(11):2637-2647. Epub 2020 Mar 4 doi: 10.1002/cncr.32795. PMID: 32129883

Recent clinical studies

Etiology

Durvalumab plus tremelimumab in advanced or metastatic soft tissue and bone sarcomas: a single-centre phase 2 trial.
Somaiah N, Conley AP, Parra ER, Lin H, Amini B, Solis Soto L, Salazar R, Barreto C, Chen H, Gite S, Haymaker C, Nassif EF, Bernatchez C, Mitra A, Livingston JA, Ravi V, Araujo DM, Benjamin R, Patel S, Zarzour MA, Sabir S, Lazar AJ, Wang WL, Daw NC, Zhou X, Roland CL, Cooper ZA, Rodriguez-Canales J, Futreal A, Soria JC, Wistuba II, Hwu P
Lancet Oncol 2022 Sep;23(9):1156-1166. Epub 2022 Aug 4 doi: 10.1016/S1470-2045(22)00392-8. PMID: 35934010
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
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
Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: A propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.
D'Ambrosio L, Touati N, Blay JY, Grignani G, Flippot R, Czarnecka AM, Piperno-Neumann S, Martin-Broto J, Sanfilippo R, Katz D, Duffaud F, Vincenzi B, Stark DP, Mazzeo F, Tuchscherer A, Chevreau C, Sherriff J, Estival A, Litière S, Sents W, Ray-Coquard I, Tolomeo F, Le Cesne A, Rutkowski P, Stacchiotti S, Kasper B, Gelderblom H, Gronchi A; European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Cancer 2020 Jun 1;126(11):2637-2647. Epub 2020 Mar 4 doi: 10.1002/cncr.32795. PMID: 32129883
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

Leiomyosarcoma: Current Clinical Management and Future Horizons.
Devaud N, Vornicova O, Abdul Razak AR, Khalili K, Demicco EG, Mitric C, Bernardini MQ, Gladdy RA
Surg Oncol Clin N Am 2022 Jul;31(3):527-546. doi: 10.1016/j.soc.2022.03.011. PMID: 35715148
What Clinical Trials Are Needed for Treatment of Leiomyosarcoma?
Kasper B, D'Ambrosio L, Davis EJ, Ingham M, Broto JM, Trent JC, van Houdt WJ, Van Tine BA
Curr Treat Options Oncol 2022 Mar;23(3):439-449. Epub 2022 Mar 11 doi: 10.1007/s11864-021-00928-y. PMID: 35275323Free 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
Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: A propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.
D'Ambrosio L, Touati N, Blay JY, Grignani G, Flippot R, Czarnecka AM, Piperno-Neumann S, Martin-Broto J, Sanfilippo R, Katz D, Duffaud F, Vincenzi B, Stark DP, Mazzeo F, Tuchscherer A, Chevreau C, Sherriff J, Estival A, Litière S, Sents W, Ray-Coquard I, Tolomeo F, Le Cesne A, Rutkowski P, Stacchiotti S, Kasper B, Gelderblom H, Gronchi A; European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Cancer 2020 Jun 1;126(11):2637-2647. Epub 2020 Mar 4 doi: 10.1002/cncr.32795. PMID: 32129883
Basic Knowledge in Soft Tissue Sarcoma.
Bourcier K, Le Cesne A, Tselikas L, Adam J, Mir O, Honore C, de Baere T
Cardiovasc Intervent Radiol 2019 Sep;42(9):1255-1261. Epub 2019 Jun 24 doi: 10.1007/s00270-019-02259-w. PMID: 31236647

Therapy

Durvalumab plus tremelimumab in advanced or metastatic soft tissue and bone sarcomas: a single-centre phase 2 trial.
Somaiah N, Conley AP, Parra ER, Lin H, Amini B, Solis Soto L, Salazar R, Barreto C, Chen H, Gite S, Haymaker C, Nassif EF, Bernatchez C, Mitra A, Livingston JA, Ravi V, Araujo DM, Benjamin R, Patel S, Zarzour MA, Sabir S, Lazar AJ, Wang WL, Daw NC, Zhou X, Roland CL, Cooper ZA, Rodriguez-Canales J, Futreal A, Soria JC, Wistuba II, Hwu P
Lancet Oncol 2022 Sep;23(9):1156-1166. Epub 2022 Aug 4 doi: 10.1016/S1470-2045(22)00392-8. PMID: 35934010
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
Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: A propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.
D'Ambrosio L, Touati N, Blay JY, Grignani G, Flippot R, Czarnecka AM, Piperno-Neumann S, Martin-Broto J, Sanfilippo R, Katz D, Duffaud F, Vincenzi B, Stark DP, Mazzeo F, Tuchscherer A, Chevreau C, Sherriff J, Estival A, Litière S, Sents W, Ray-Coquard I, Tolomeo F, Le Cesne A, Rutkowski P, Stacchiotti S, Kasper B, Gelderblom H, Gronchi A; European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Cancer 2020 Jun 1;126(11):2637-2647. Epub 2020 Mar 4 doi: 10.1002/cncr.32795. PMID: 32129883
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
Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial.
Schöffski P, Chawla S, Maki RG, Italiano A, Gelderblom H, Choy E, Grignani G, Camargo V, Bauer S, Rha SY, Blay JY, Hohenberger P, D'Adamo D, Guo M, Chmielowski B, Le Cesne A, Demetri GD, Patel SR
Lancet 2016 Apr 16;387(10028):1629-37. Epub 2016 Feb 10 doi: 10.1016/S0140-6736(15)01283-0. PMID: 26874885

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
What Clinical Trials Are Needed for Treatment of Leiomyosarcoma?
Kasper B, D'Ambrosio L, Davis EJ, Ingham M, Broto JM, Trent JC, van Houdt WJ, Van Tine BA
Curr Treat Options Oncol 2022 Mar;23(3):439-449. Epub 2022 Mar 11 doi: 10.1007/s11864-021-00928-y. PMID: 35275323Free PMC Article
Basic Knowledge in Soft Tissue Sarcoma.
Bourcier K, Le Cesne A, Tselikas L, Adam J, Mir O, Honore C, de Baere T
Cardiovasc Intervent Radiol 2019 Sep;42(9):1255-1261. Epub 2019 Jun 24 doi: 10.1007/s00270-019-02259-w. PMID: 31236647
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
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

Efficacy thresholds for clinical trials with advanced or metastatic leiomyosarcoma patients: A European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group meta-analysis based on a literature review for soft-tissue sarcomas.
Kantidakis G, Litière S, Neven A, Vinches M, Judson I, Schöffski P, Wardelmann E, Stacchiotti S, D'Ambrosio L, Marréaud S, van der Graaf WTA, Kasper B, Fiocco M, Gelderblom H
Eur J Cancer 2021 Sep;154:253-268. Epub 2021 Jul 20 doi: 10.1016/j.ejca.2021.06.025. PMID: 34298376
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Doxorubicin plus dacarbazine, doxorubicin plus ifosfamide, or doxorubicin alone as a first-line treatment for advanced leiomyosarcoma: A propensity score matching analysis from the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.
D'Ambrosio L, Touati N, Blay JY, Grignani G, Flippot R, Czarnecka AM, Piperno-Neumann S, Martin-Broto J, Sanfilippo R, Katz D, Duffaud F, Vincenzi B, Stark DP, Mazzeo F, Tuchscherer A, Chevreau C, Sherriff J, Estival A, Litière S, Sents W, Ray-Coquard I, Tolomeo F, Le Cesne A, Rutkowski P, Stacchiotti S, Kasper B, Gelderblom H, Gronchi A; European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group
Cancer 2020 Jun 1;126(11):2637-2647. Epub 2020 Mar 4 doi: 10.1002/cncr.32795. PMID: 32129883
Cutaneous leiomyosarcoma.
Porter CJ, Januszkiewicz JS
Plast Reconstr Surg 2002 Mar;109(3):964-7. doi: 10.1097/00006534-200203000-00024. PMID: 11884817
Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: a study by the EORTC Soft Tissue and Bone Sarcoma Group.
Judson I, Radford JA, Harris M, Blay JY, van Hoesel Q, le Cesne A, van Oosterom AT, Clemons MJ, Kamby C, Hermans C, Whittaker J, Donato di Paola E, Verweij J, Nielsen S
Eur J Cancer 2001 May;37(7):870-7. doi: 10.1016/s0959-8049(01)00050-8. PMID: 11313175

Recent systematic reviews

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
Immune checkpoint inhibitors in treatment of soft-tissue sarcoma: A systematic review and meta-analysis.
Saerens M, Brusselaers N, Rottey S, Decruyenaere A, Creytens D, Lapeire L
Eur J Cancer 2021 Jul;152:165-182. Epub 2021 Jun 6 doi: 10.1016/j.ejca.2021.04.034. PMID: 34107450
Gemcitabine-based chemotherapy in sarcomas: A systematic review of published trials.
Ducoulombier A, Cousin S, Kotecki N, Penel N
Crit Rev Oncol Hematol 2016 Feb;98:73-80. Epub 2015 Nov 3 doi: 10.1016/j.critrevonc.2015.10.020. PMID: 26555460

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