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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

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
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
Soft tissue sarcomas, a look into the future: different treatments for different subtypes.
Reichardt P
Future Oncol 2014 Jun;10(8 Suppl):s19-27. doi: 10.2217/fon.14.116. PMID: 25048045
Phase II study of sunitinib malate, a multitargeted tyrosine kinase inhibitor in patients with relapsed or refractory soft tissue sarcomas. Focus on three prevalent histologies: leiomyosarcoma, liposarcoma and malignant fibrous histiocytoma.
Mahmood ST, Agresta S, Vigil CE, Zhao X, Han G, D'Amato G, Calitri CE, Dean M, Garrett C, Schell MJ, Antonia S, Chiappori A
Int J Cancer 2011 Oct 15;129(8):1963-9. Epub 2011 Mar 25 doi: 10.1002/ijc.25843. PMID: 21154746Free PMC Article
New therapeutic strategies for soft tissue sarcomas.
von Mehren M
Curr Treat Options Oncol 2003 Dec;4(6):441-51. doi: 10.1007/s11864-003-0045-4. PMID: 14585225

Diagnosis

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
Soft tissue sarcomas, a look into the future: different treatments for different subtypes.
Reichardt P
Future Oncol 2014 Jun;10(8 Suppl):s19-27. doi: 10.2217/fon.14.116. PMID: 25048045
Misdiagnosis of soft tissue sarcomas of the lower limb associated with deep venous thrombosis: report of two cases and review of the literature.
Perisano C, Maffulli N, Colelli P, Marzetti E, Panni AS, Maccauro G
BMC Musculoskelet Disord 2013 Feb 19;14:64. doi: 10.1186/1471-2474-14-64. PMID: 23421389Free PMC Article
Diagnosis of gastrointestinal stromal tumors from minute specimens: cytomorphology, immunohistochemistry, and molecular diagnostic findings.
Layfield LJ, Wallander ML
Diagn Cytopathol 2012 Jun;40(6):484-90. Epub 2012 Feb 20 doi: 10.1002/dc.22838. PMID: 22351482
New therapeutic strategies for soft tissue sarcomas.
von Mehren M
Curr Treat Options Oncol 2003 Dec;4(6):441-51. doi: 10.1007/s11864-003-0045-4. PMID: 14585225

Therapy

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
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
New therapeutic strategies for soft tissue sarcomas.
von Mehren M
Curr Treat Options Oncol 2003 Dec;4(6):441-51. doi: 10.1007/s11864-003-0045-4. PMID: 14585225

Prognosis

Safety and Efficacy of Anlotinib, a Multikinase Angiogenesis Inhibitor, in Patients with Refractory Metastatic Soft-Tissue Sarcoma.
Chi Y, Fang Z, Hong X, Yao Y, Sun P, Wang G, Du F, Sun Y, Wu Q, Qu G, Wang S, Song J, Yu J, Lu Y, Zhu X, Niu X, He Z, Wang J, Yu H, Cai J
Clin Cancer Res 2018 Nov 1;24(21):5233-5238. Epub 2018 Jun 12 doi: 10.1158/1078-0432.CCR-17-3766. PMID: 29895706
Diagnosis of gastrointestinal stromal tumors from minute specimens: cytomorphology, immunohistochemistry, and molecular diagnostic findings.
Layfield LJ, Wallander ML
Diagn Cytopathol 2012 Jun;40(6):484-90. Epub 2012 Feb 20 doi: 10.1002/dc.22838. PMID: 22351482
A phase II trial of thalidomide in patients with refractory leiomyosarcoma of the uterus and correlation with biomarkers of angiogenesis: a gynecologic oncology group study.
McMeekin DS, Sill MW, Darcy KM, Stearns-Kurosawa DJ, Webster K, Waggoner S, Benbrook D
Gynecol Oncol 2007 Sep;106(3):596-603. Epub 2007 Jun 27 doi: 10.1016/j.ygyno.2007.05.013. PMID: 17597196
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
New therapeutic strategies for soft tissue sarcomas.
von Mehren M
Curr Treat Options Oncol 2003 Dec;4(6):441-51. doi: 10.1007/s11864-003-0045-4. PMID: 14585225

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 of Pazopanib With or Without Gemcitabine in Patients With Anthracycline- and/or Ifosfamide-Refractory Soft Tissue Sarcoma: Final Results of the PAPAGEMO Phase 2 Randomized Clinical Trial.
Schmoll HJ, Lindner LH, Reichardt P, Heißner K, Kopp HG, Kessler T, Mayer-Steinacker R, Rüssel J, Egerer G, Crysandt M, Kasper B, Niederwieser D, Kunitz A, Eigendorff E, Petersen I, Steighardt J, Cygon F, Meinert F, Stein A
JAMA Oncol 2021 Feb 1;7(2):255-262. doi: 10.1001/jamaoncol.2020.6564. PMID: 33355646Free 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
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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