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Refractory Malignant Thymoma

MedGen UID:
1644175
•Concept ID:
C4688372
•
Neoplastic Process

Definition

Malignant thymoma that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Malignant Thymoma

Professional guidelines

PubMed

Myasthenia gravis-Pathophysiology, diagnosis, and treatment.
Tannemaat MR, Huijbers MG, Verschuuren JJGM
Handb Clin Neurol 2024;200:283-305. doi: 10.1016/B978-0-12-823912-4.00026-8. PMID: 38494283
Eculizumab treatment for myasthenia gravis subgroups: 2021 update.
Jiao L, Li H, Guo S
J Neuroimmunol 2022 Jan 15;362:577767. Epub 2021 Nov 18 doi: 10.1016/j.jneuroim.2021.577767. PMID: 34823117
Novel Agents in the Treatment of Thymic Malignancies.
Merveilleux du Vignaux C, Maury JM, Girard N
Curr Treat Options Oncol 2017 Aug 10;18(9):52. doi: 10.1007/s11864-017-0495-8. PMID: 28795288

Recent clinical studies

Etiology

Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis: The RINOMAX Randomized Clinical Trial.
Piehl F, Eriksson-Dufva A, Budzianowska A, Feresiadou A, Hansson W, Hietala MA, Håkansson I, Johansson R, Jons D, Kmezic I, Lindberg C, Lindh J, Lundin F, Nygren I, Punga AR, Press R, Samuelsson K, Sundström P, Wickberg O, Brauner S, Frisell T
JAMA Neurol 2022 Nov 1;79(11):1105-1112. doi: 10.1001/jamaneurol.2022.2887. PMID: 36121672Free PMC Article
Immunobiology of Thymic Epithelial Tumors: Implications for Immunotherapy with Immune Checkpoint Inhibitors.
Tateo V, Manuzzi L, De Giglio A, Parisi C, Lamberti G, Campana D, Pantaleo MA
Int J Mol Sci 2020 Nov 28;21(23) doi: 10.3390/ijms21239056. PMID: 33260538Free PMC Article
Refractory myasthenia gravis: Characteristics of a portuguese cohort.
Santos E, Bettencourt A, Duarte S, Gabriel D, Oliveira V, da Silva AM, Costa PP, Lopes C, Gonçalves G, da Silva BM, Leite MI
Muscle Nerve 2019 Aug;60(2):188-191. Epub 2019 May 15 doi: 10.1002/mus.26507. PMID: 31050006
Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.
Howard JF Jr, Utsugisawa K, Benatar M, Murai H, Barohn RJ, Illa I, Jacob S, Vissing J, Burns TM, Kissel JT, Muppidi S, Nowak RJ, O'Brien F, Wang JJ, Mantegazza R; REGAIN Study Group
Lancet Neurol 2017 Dec;16(12):976-986. Epub 2017 Oct 20 doi: 10.1016/S1474-4422(17)30369-1. PMID: 29066163
Tumor molecular profiling in the treatment of refractory cancers.
Birendra KC, Afzal MZ, Sochaki A, Wentland KA, Chang R, Singh S, O'Rourke T
J Exp Ther Oncol 2015;11(1):27-32. PMID: 26259387

Diagnosis

Myasthenia gravis-Pathophysiology, diagnosis, and treatment.
Tannemaat MR, Huijbers MG, Verschuuren JJGM
Handb Clin Neurol 2024;200:283-305. doi: 10.1016/B978-0-12-823912-4.00026-8. PMID: 38494283
Refractory Morvan syndrome responsive to rituximab: a case report and review of the literature.
Nawfal O, Agha M, Makki A, Beydoun A
Neuromuscul Disord 2022 Aug;32(8):682-686. Epub 2022 Jun 2 doi: 10.1016/j.nmd.2022.06.001. PMID: 35715317
Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.
Howard JF Jr, Utsugisawa K, Benatar M, Murai H, Barohn RJ, Illa I, Jacob S, Vissing J, Burns TM, Kissel JT, Muppidi S, Nowak RJ, O'Brien F, Wang JJ, Mantegazza R; REGAIN Study Group
Lancet Neurol 2017 Dec;16(12):976-986. Epub 2017 Oct 20 doi: 10.1016/S1474-4422(17)30369-1. PMID: 29066163
Novel Agents in the Treatment of Thymic Malignancies.
Merveilleux du Vignaux C, Maury JM, Girard N
Curr Treat Options Oncol 2017 Aug 10;18(9):52. doi: 10.1007/s11864-017-0495-8. PMID: 28795288
Tumor molecular profiling in the treatment of refractory cancers.
Birendra KC, Afzal MZ, Sochaki A, Wentland KA, Chang R, Singh S, O'Rourke T
J Exp Ther Oncol 2015;11(1):27-32. PMID: 26259387

Therapy

Novel uses of complement inhibitors in myasthenia gravis-Two case reports.
Zadeh S, Price H, Drews R, Bouffard MA, Young LH, Narayanaswami P
Muscle Nerve 2024 Mar;69(3):368-372. Epub 2024 Jan 11 doi: 10.1002/mus.28037. PMID: 38205840
Eculizumab treatment for myasthenia gravis subgroups: 2021 update.
Jiao L, Li H, Guo S
J Neuroimmunol 2022 Jan 15;362:577767. Epub 2021 Nov 18 doi: 10.1016/j.jneuroim.2021.577767. PMID: 34823117
Immunobiology of Thymic Epithelial Tumors: Implications for Immunotherapy with Immune Checkpoint Inhibitors.
Tateo V, Manuzzi L, De Giglio A, Parisi C, Lamberti G, Campana D, Pantaleo MA
Int J Mol Sci 2020 Nov 28;21(23) doi: 10.3390/ijms21239056. PMID: 33260538Free PMC Article
Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.
Howard JF Jr, Utsugisawa K, Benatar M, Murai H, Barohn RJ, Illa I, Jacob S, Vissing J, Burns TM, Kissel JT, Muppidi S, Nowak RJ, O'Brien F, Wang JJ, Mantegazza R; REGAIN Study Group
Lancet Neurol 2017 Dec;16(12):976-986. Epub 2017 Oct 20 doi: 10.1016/S1474-4422(17)30369-1. PMID: 29066163
Novel Agents in the Treatment of Thymic Malignancies.
Merveilleux du Vignaux C, Maury JM, Girard N
Curr Treat Options Oncol 2017 Aug 10;18(9):52. doi: 10.1007/s11864-017-0495-8. PMID: 28795288

Prognosis

The efficacy of small molecule anti-angiogenic drugs in previously treated Thymic carcinoma.
Guan Y, Gu X, Si J, Xiang J, Wei J, Hao Y, Wang W, Sun Y
BMC Cancer 2023 Jan 5;23(1):16. doi: 10.1186/s12885-022-10448-z. PMID: 36604688Free PMC Article
Fatal adverse events in two thymoma patients treated with anti-PD-1 immune check point inhibitor and literature review.
Konstantina T, Konstantinos R, Anastasios K, Anastasia M, Eleni L, Ioannis S, Sofia A, Dimitris M
Lung Cancer 2019 Sep;135:29-32. Epub 2019 Jul 9 doi: 10.1016/j.lungcan.2019.06.015. PMID: 31446999
Neoantigen identification strategies enable personalized immunotherapy in refractory solid tumors.
Chen F, Zou Z, Du J, Su S, Shao J, Meng F, Yang J, Xu Q, Ding N, Yang Y, Liu Q, Wang Q, Sun Z, Zhou S, Du S, Wei J, Liu B
J Clin Invest 2019 May 1;129(5):2056-2070. Epub 2019 Mar 5 doi: 10.1172/JCI99538. PMID: 30835255Free PMC Article
Tumor molecular profiling in the treatment of refractory cancers.
Birendra KC, Afzal MZ, Sochaki A, Wentland KA, Chang R, Singh S, O'Rourke T
J Exp Ther Oncol 2015;11(1):27-32. PMID: 26259387
Targeted therapy for thymic epithelial tumors: a new horizon? Review of the literature and two cases reports.
Simonelli M, Zucali PA, Suter MB, Lorenzi E, Rubino L, Fatuzzo G, Alloisio M, Santoro A
Future Oncol 2015;11(8):1223-32. doi: 10.2217/fon.14.318. PMID: 25832879

Clinical prediction guides

Myasthenia gravis-Pathophysiology, diagnosis, and treatment.
Tannemaat MR, Huijbers MG, Verschuuren JJGM
Handb Clin Neurol 2024;200:283-305. doi: 10.1016/B978-0-12-823912-4.00026-8. PMID: 38494283
Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis: The RINOMAX Randomized Clinical Trial.
Piehl F, Eriksson-Dufva A, Budzianowska A, Feresiadou A, Hansson W, Hietala MA, Håkansson I, Johansson R, Jons D, Kmezic I, Lindberg C, Lindh J, Lundin F, Nygren I, Punga AR, Press R, Samuelsson K, Sundström P, Wickberg O, Brauner S, Frisell T
JAMA Neurol 2022 Nov 1;79(11):1105-1112. doi: 10.1001/jamaneurol.2022.2887. PMID: 36121672Free PMC Article
Immunobiology of Thymic Epithelial Tumors: Implications for Immunotherapy with Immune Checkpoint Inhibitors.
Tateo V, Manuzzi L, De Giglio A, Parisi C, Lamberti G, Campana D, Pantaleo MA
Int J Mol Sci 2020 Nov 28;21(23) doi: 10.3390/ijms21239056. PMID: 33260538Free PMC Article
Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study.
Howard JF Jr, Utsugisawa K, Benatar M, Murai H, Barohn RJ, Illa I, Jacob S, Vissing J, Burns TM, Kissel JT, Muppidi S, Nowak RJ, O'Brien F, Wang JJ, Mantegazza R; REGAIN Study Group
Lancet Neurol 2017 Dec;16(12):976-986. Epub 2017 Oct 20 doi: 10.1016/S1474-4422(17)30369-1. PMID: 29066163
Tumor molecular profiling in the treatment of refractory cancers.
Birendra KC, Afzal MZ, Sochaki A, Wentland KA, Chang R, Singh S, O'Rourke T
J Exp Ther Oncol 2015;11(1):27-32. PMID: 26259387

Recent systematic reviews

Efficacy and safety of treatments for advanced thymic carcinoma after failure of first-line platinum-based chemotherapy: A systematic literature review and meta-analysis.
Arunachalam A, Zhang I, Zhao B, Frederickson AM, Catherine Pietanza M
Lung Cancer 2023 Feb;176:132-139. Epub 2023 Jan 2 doi: 10.1016/j.lungcan.2023.01.003. PMID: 36638588
Systematic review of the clinical spectrum of CASPR2 antibody syndrome.
Boyko M, Au KLK, Casault C, de Robles P, Pfeffer G
J Neurol 2020 Apr;267(4):1137-1146. Epub 2020 Jan 7 doi: 10.1007/s00415-019-09686-2. PMID: 31912210

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