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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
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

Recent clinical studies

Etiology

Reduced Number of Thymoma CTLA4-Positive Cells Is Associated With a Higher Probability of Developing Myasthenia Gravis.
Álvarez-Velasco R, Dols-Icardo O, El Bounasri S, López-Vilaró L, Trujillo JC, Reyes-Leiva D, Suárez-Calvet X, Cortés-Vicente E, Illa I, Gallardo E
Neurol Neuroimmunol Neuroinflamm 2023 Mar;10(2) Epub 2023 Jan 25 doi: 10.1212/NXI.0000000000200085. PMID: 36697230Free PMC Article
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
Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.
Álvarez-Velasco R, Gutiérrez-Gutiérrez G, Trujillo JC, Martínez E, Segovia S, Arribas-Velasco M, Fernández G, Paradas C, Vélez-Gómez B, Casasnovas C, Nedkova V, Guerrero-Sola A, Ramos-Fransi A, Martínez-Piñeiro A, Pardo J, Sevilla T, Gómez-Caravaca MT, López de Munain A, Jericó I, Pelayo-Negro AL, Martín MA, Morgado Y, Mendoza MD, Pérez-Pérez H, Rojas-García R, Turon-Sans J, Querol L, Gallardo E, Illa I, Cortés-Vicente E
Eur J Neurol 2021 Jun;28(6):2083-2091. Epub 2021 Mar 30 doi: 10.1111/ene.14820. PMID: 33721382
Paraneoplastic and Therapy-Related Immune Complications in Thymic Malignancies.
Lippner EA, Lewis DB, Robinson WH, Katsumoto TR
Curr Treat Options Oncol 2019 Jun 22;20(7):62. doi: 10.1007/s11864-019-0661-2. PMID: 31227926
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

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
T-Lymphoblastic Leukemia/Lymphoma and Thymoma: A Case Report and Review of the Literature of a Rare Association.
Mizrahi N, Kugler E, Hayman L, Yeshurun M, Grossman A, Raanani P, Wolach O
Acta Haematol 2022;145(1):106-111. Epub 2021 Sep 17 doi: 10.1159/000519033. PMID: 34537764
Paraneoplastic and Therapy-Related Immune Complications in Thymic Malignancies.
Lippner EA, Lewis DB, Robinson WH, Katsumoto TR
Curr Treat Options Oncol 2019 Jun 22;20(7):62. doi: 10.1007/s11864-019-0661-2. PMID: 31227926
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
Limbic encephalitis and variants: classification, diagnosis and treatment.
Tüzün E, Dalmau J
Neurologist 2007 Sep;13(5):261-71. doi: 10.1097/NRL.0b013e31813e34a5. PMID: 17848866

Therapy

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
Thymic malignancies: emerging systemic therapies.
Girard N
Curr Opin Oncol 2019 Sep;31(5):454-460. doi: 10.1097/CCO.0000000000000564. PMID: 31394557
Paraneoplastic and Therapy-Related Immune Complications in Thymic Malignancies.
Lippner EA, Lewis DB, Robinson WH, Katsumoto TR
Curr Treat Options Oncol 2019 Jun 22;20(7):62. doi: 10.1007/s11864-019-0661-2. PMID: 31227926
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
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

Prognosis

Reduced Number of Thymoma CTLA4-Positive Cells Is Associated With a Higher Probability of Developing Myasthenia Gravis.
Álvarez-Velasco R, Dols-Icardo O, El Bounasri S, López-Vilaró L, Trujillo JC, Reyes-Leiva D, Suárez-Calvet X, Cortés-Vicente E, Illa I, Gallardo E
Neurol Neuroimmunol Neuroinflamm 2023 Mar;10(2) Epub 2023 Jan 25 doi: 10.1212/NXI.0000000000200085. PMID: 36697230Free PMC Article
T-Lymphoblastic Leukemia/Lymphoma and Thymoma: A Case Report and Review of the Literature of a Rare Association.
Mizrahi N, Kugler E, Hayman L, Yeshurun M, Grossman A, Raanani P, Wolach O
Acta Haematol 2022;145(1):106-111. Epub 2021 Sep 17 doi: 10.1159/000519033. PMID: 34537764
Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.
Álvarez-Velasco R, Gutiérrez-Gutiérrez G, Trujillo JC, Martínez E, Segovia S, Arribas-Velasco M, Fernández G, Paradas C, Vélez-Gómez B, Casasnovas C, Nedkova V, Guerrero-Sola A, Ramos-Fransi A, Martínez-Piñeiro A, Pardo J, Sevilla T, Gómez-Caravaca MT, López de Munain A, Jericó I, Pelayo-Negro AL, Martín MA, Morgado Y, Mendoza MD, Pérez-Pérez H, Rojas-García R, Turon-Sans J, Querol L, Gallardo E, Illa I, Cortés-Vicente E
Eur J Neurol 2021 Jun;28(6):2083-2091. Epub 2021 Mar 30 doi: 10.1111/ene.14820. PMID: 33721382
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
Sunitinib in patients with chemotherapy-refractory thymoma and thymic carcinoma: an open-label phase 2 trial.
Thomas A, Rajan A, Berman A, Tomita Y, Brzezniak C, Lee MJ, Lee S, Ling A, Spittler AJ, Carter CA, Guha U, Wang Y, Szabo E, Meltzer P, Steinberg SM, Trepel JB, Loehrer PJ, Giaccone G
Lancet Oncol 2015 Feb;16(2):177-86. Epub 2015 Jan 13 doi: 10.1016/S1470-2045(14)71181-7. PMID: 25592632Free PMC Article

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
Reduced Number of Thymoma CTLA4-Positive Cells Is Associated With a Higher Probability of Developing Myasthenia Gravis.
Álvarez-Velasco R, Dols-Icardo O, El Bounasri S, López-Vilaró L, Trujillo JC, Reyes-Leiva D, Suárez-Calvet X, Cortés-Vicente E, Illa I, Gallardo E
Neurol Neuroimmunol Neuroinflamm 2023 Mar;10(2) Epub 2023 Jan 25 doi: 10.1212/NXI.0000000000200085. PMID: 36697230Free PMC Article
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
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
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

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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