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Refractory Leiomyosarcoma

MedGen UID:
1652428
•Concept ID:
C4724965
•
Neoplastic Process

Definition

Leiomyosarcoma that does not respond to treatment. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Clinical Benefit in Response to Palbociclib Treatment in Refractory Uterine Leiomyosarcomas with a Common CDKN2A Alteration.
Elvin JA, Gay LM, Ort R, Shuluk J, Long J, Shelley L, Lee R, Chalmers ZR, Frampton GM, Ali SM, Schrock AB, Miller VA, Stephens PJ, Ross JS, Frank R
Oncologist 2017 Apr;22(4):416-421. Epub 2017 Mar 10 doi: 10.1634/theoncologist.2016-0310. PMID: 28283584Free PMC Article
A retrospective chart review of drug treatment patterns and clinical outcomes among patients with metastatic or recurrent soft tissue sarcoma refractory to one or more prior chemotherapy treatments.
Wagner MJ, Amodu LI, Duh MS, Korves C, Solleza F, Manson SC, Diaz J, Neary MP, Demetri GD
BMC Cancer 2015 Mar 25;15:175. doi: 10.1186/s12885-015-1182-4. PMID: 25885530Free PMC Article
Gemcitabine in the treatment of soft tissue sarcomas.
Bauer S, Seeber S, Schütte J
Onkologie 2004 Apr;27(2):180-6. doi: 10.1159/000076910. PMID: 15138353

Recent clinical studies

Etiology

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
Efficacy and safety of regorafenib compared to placebo and to post-cross-over regorafenib in advanced non-adipocytic soft tissue sarcoma.
Brodowicz T, Mir O, Wallet J, Italiano A, Blay JY, Bertucci F, Eisterer W, Chevreau C, Piperno-Neumann S, Bompas E, Ryckewaert T, Liegl-Antzwager B, Thery J, Penel N, Le Cesne A, Le Deley MC
Eur J Cancer 2018 Aug;99:28-36. Epub 2018 Jun 11 doi: 10.1016/j.ejca.2018.05.008. PMID: 29902612
A retrospective chart review of drug treatment patterns and clinical outcomes among patients with metastatic or recurrent soft tissue sarcoma refractory to one or more prior chemotherapy treatments.
Wagner MJ, Amodu LI, Duh MS, Korves C, Solleza F, Manson SC, Diaz J, Neary MP, Demetri GD
BMC Cancer 2015 Mar 25;15:175. doi: 10.1186/s12885-015-1182-4. PMID: 25885530Free PMC Article
Establishment and operation of a Good Manufacturing Practice-compliant allogeneic Epstein-Barr virus (EBV)-specific cytotoxic cell bank for the treatment of EBV-associated lymphoproliferative disease.
Vickers MA, Wilkie GM, Robinson N, Rivera N, Haque T, Crawford DH, Barry J, Fraser N, Turner DM, Robertson V, Dyer P, Flanagan P, Newlands HR, Campbell J, Turner ML
Br J Haematol 2014 Nov;167(3):402-10. Epub 2014 Jul 26 doi: 10.1111/bjh.13051. PMID: 25066775Free PMC Article
Gemcitabine in soft tissue or bone sarcoma resistant to standard chemotherapy: a phase II study.
Merimsky O, Meller I, Flusser G, Kollender Y, Issakov J, Weil-Ben-Arush M, Fenig E, Neuman G, Sapir D, Ariad S, Inbar M
Cancer Chemother Pharmacol 2000;45(2):177-81. doi: 10.1007/s002800050027. PMID: 10663634

Diagnosis

LP-184, a Novel Acylfulvene Molecule, Exhibits Anticancer Activity against Diverse Solid Tumors with Homologous Recombination Deficiency.
Kulkarni A, Zhou J, Biyani N, Kathad U, Banerjee PP, Srivastava S, Prucsi Z, Solarczyk K, Bhatia K, Ewesuedo RB, Sharma P
Cancer Res Commun 2024 May 6;4(5):1199-1210. doi: 10.1158/2767-9764.CRC-23-0554. PMID: 38630886Free 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
REGOSARC: Regorafenib versus placebo in doxorubicin-refractory soft-tissue sarcoma-A quality-adjusted time without symptoms of progression or toxicity analysis.
Berry V, Basson L, Bogart E, Mir O, Blay JY, Italiano A, Bertucci F, Chevreau C, Clisant-Delaine S, Liegl-Antzager B, Tresch-Bruneel E, Wallet J, Taieb S, Decoupigny E, Le Cesne A, Brodowicz T, Penel N
Cancer 2017 Jun 15;123(12):2294-2302. Epub 2017 Mar 10 doi: 10.1002/cncr.30661. PMID: 28295221Free PMC Article
A retrospective chart review of drug treatment patterns and clinical outcomes among patients with metastatic or recurrent soft tissue sarcoma refractory to one or more prior chemotherapy treatments.
Wagner MJ, Amodu LI, Duh MS, Korves C, Solleza F, Manson SC, Diaz J, Neary MP, Demetri GD
BMC Cancer 2015 Mar 25;15:175. doi: 10.1186/s12885-015-1182-4. PMID: 25885530Free PMC Article
Primary leiomyosarcoma of saphenous vein presenting as deep venous thrombosis.
Fremed DI, Faries PL, Schanzer HR, Marin ML, Ting W
Vascular 2014 Dec;22(6):450-3. Epub 2013 Dec 17 doi: 10.1177/1708538113516446. PMID: 24347138

Therapy

LP-184, a Novel Acylfulvene Molecule, Exhibits Anticancer Activity against Diverse Solid Tumors with Homologous Recombination Deficiency.
Kulkarni A, Zhou J, Biyani N, Kathad U, Banerjee PP, Srivastava S, Prucsi Z, Solarczyk K, Bhatia K, Ewesuedo RB, Sharma P
Cancer Res Commun 2024 May 6;4(5):1199-1210. doi: 10.1158/2767-9764.CRC-23-0554. PMID: 38630886Free PMC Article
Molecular analysis for refractory rare cancers: Sequencing battle continues - learnings for the MOSCATO-01 study.
Debien V, Vignot S, Massard C, Malouf G, Hollebecque A, Scoazec JY, Michiels S, Verlingue L
Crit Rev Oncol Hematol 2023 Jan;181:103888. Epub 2022 Nov 29 doi: 10.1016/j.critrevonc.2022.103888. PMID: 36460264
Effect of Capivasertib in Patients With an AKT1 E17K-Mutated Tumor: NCI-MATCH Subprotocol EAY131-Y Nonrandomized Trial.
Kalinsky K, Hong F, McCourt CK, Sachdev JC, Mitchell EP, Zwiebel JA, Doyle LA, McShane LM, Li S, Gray RJ, Rubinstein LV, Patton D, Williams PM, Hamilton SR, Conley BA, O'Dwyer PJ, Harris LN, Arteaga CL, Chen AP, Flaherty KT
JAMA Oncol 2021 Feb 1;7(2):271-278. doi: 10.1001/jamaoncol.2020.6741. PMID: 33377972Free PMC Article
Primary intracranial leiomyosarcoma in an HIV-infected patient.
Sivendran S, Vidal CI, Barginear MF
Int J Clin Oncol 2011 Feb;16(1):63-6. Epub 2010 Jul 30 doi: 10.1007/s10147-010-0110-5. PMID: 20676716
Gemcitabine in the treatment of soft tissue sarcomas.
Bauer S, Seeber S, Schütte J
Onkologie 2004 Apr;27(2):180-6. doi: 10.1159/000076910. PMID: 15138353

Prognosis

Efficacy and safety of regorafenib compared to placebo and to post-cross-over regorafenib in advanced non-adipocytic soft tissue sarcoma.
Brodowicz T, Mir O, Wallet J, Italiano A, Blay JY, Bertucci F, Eisterer W, Chevreau C, Piperno-Neumann S, Bompas E, Ryckewaert T, Liegl-Antzwager B, Thery J, Penel N, Le Cesne A, Le Deley MC
Eur J Cancer 2018 Aug;99:28-36. Epub 2018 Jun 11 doi: 10.1016/j.ejca.2018.05.008. PMID: 29902612
Long-lasting activity of trabectedin in refractory uterine leiomyosarcoma: a case report.
Bongiovanni A, Riva N, Ricci M, Mercatali L, Liverani C, La Manna F, De Vita A, Cavaliere D, Pieri F, Oboldi D, Verdecchia GM, Amadori D, Ibrahim T
BMC Cancer 2015 Dec 22;15:998. doi: 10.1186/s12885-015-2038-7. PMID: 26695071Free PMC Article
A retrospective chart review of drug treatment patterns and clinical outcomes among patients with metastatic or recurrent soft tissue sarcoma refractory to one or more prior chemotherapy treatments.
Wagner MJ, Amodu LI, Duh MS, Korves C, Solleza F, Manson SC, Diaz J, Neary MP, Demetri GD
BMC Cancer 2015 Mar 25;15:175. doi: 10.1186/s12885-015-1182-4. PMID: 25885530Free PMC Article
Gemcitabine in the treatment of soft tissue sarcomas.
Bauer S, Seeber S, Schütte J
Onkologie 2004 Apr;27(2):180-6. doi: 10.1159/000076910. PMID: 15138353
Gemcitabine in soft tissue or bone sarcoma resistant to standard chemotherapy: a phase II study.
Merimsky O, Meller I, Flusser G, Kollender Y, Issakov J, Weil-Ben-Arush M, Fenig E, Neuman G, Sapir D, Ariad S, Inbar M
Cancer Chemother Pharmacol 2000;45(2):177-81. doi: 10.1007/s002800050027. PMID: 10663634

Clinical prediction guides

Phase I Study of mTORC1/2 Inhibitor Sapanisertib (CB-228/TAK-228) in Combination with Metformin in Patients with mTOR/AKT/PI3K Pathway Alterations and Advanced Solid Malignancies.
Subbiah V, Coleman N, Piha-Paul SA, Tsimberidou AM, Janku F, Rodon J, Pant S, Dumbrava EEI, Fu S, Hong DS, Zhang S, Sun M, Jiang Y, Roszik J, Song J, Yuan Y, Meric-Bernstam F, Naing A
Cancer Res Commun 2024 Feb 12;4(2):378-387. doi: 10.1158/2767-9764.CRC-22-0260. PMID: 38126764Free PMC Article
Regorafenib in advanced solitary fibrous tumour: Results from an exploratory phase II clinical study.
Stacchiotti S, Baldi GG, Frezza AM, Morosi C, Greco FG, Collini P, Barisella M, Dagrada GP, Zaffaroni N, Pasquali S, Gronchi A, Huang P, Ingrosso M, Tinè G, Miceli R, Casali PG
Eur J Cancer 2023 Dec;195:113391. Epub 2023 Oct 18 doi: 10.1016/j.ejca.2023.113391. PMID: 37918286
Efficacy and safety of regorafenib compared to placebo and to post-cross-over regorafenib in advanced non-adipocytic soft tissue sarcoma.
Brodowicz T, Mir O, Wallet J, Italiano A, Blay JY, Bertucci F, Eisterer W, Chevreau C, Piperno-Neumann S, Bompas E, Ryckewaert T, Liegl-Antzwager B, Thery J, Penel N, Le Cesne A, Le Deley MC
Eur J Cancer 2018 Aug;99:28-36. Epub 2018 Jun 11 doi: 10.1016/j.ejca.2018.05.008. PMID: 29902612
Gemcitabine in the treatment of soft tissue sarcomas.
Bauer S, Seeber S, Schütte J
Onkologie 2004 Apr;27(2):180-6. doi: 10.1159/000076910. PMID: 15138353
Development of cyclin-dependent kinase modulators as novel therapeutic approaches for hematological malignancies.
Senderowicz AM
Leukemia 2001 Jan;15(1):1-9. doi: 10.1038/sj.leu.2401994. PMID: 11243375

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