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Metaplastic Thymoma

MedGen UID:
317905
•Concept ID:
C1708993
•
Neoplastic Process
Synonyms: Biphasic Thymoma; Low Grade Thymic Metaplastic Carcinoma; Metaplastic thymoma; Mixed Polygonal and Spindle Cell Type Thymoma; Thymoma with Pseudosarcomatous Stroma
SNOMED CT: Metaplastic thymoma (726421003)

Definition

A rare type of well circumscribed or encapsulated thymoma with a biphasic architecture, consisting of epithelial cell islands and bundles of spindle cells. The vast majority of reported cases had a benign clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetaplastic Thymoma

Professional guidelines

PubMed

Significance of YAP1-MAML2 rearrangement and GTF2I mutation in the diagnosis and differential diagnosis of metaplastic thymoma.
Wang M, Xu H, Han Q, Wang L
Ann Med 2023;55(2):2237040. doi: 10.1080/07853890.2023.2237040. PMID: 37489594Free PMC Article

Recent clinical studies

Etiology

Significance of YAP1-MAML2 rearrangement and GTF2I mutation in the diagnosis and differential diagnosis of metaplastic thymoma.
Wang M, Xu H, Han Q, Wang L
Ann Med 2023;55(2):2237040. doi: 10.1080/07853890.2023.2237040. PMID: 37489594Free PMC Article
D2-40 Antibody is a Specific Marker for B2 Thymomas.
Szolkowska M, Langfort R, Winiarski S, Rudzinski P, Giedronowicz D, Szczepulska-Wojcik E, Ronduda P, Maksymiuk B, Orlowski T
Appl Immunohistochem Mol Morphol 2017 Jul;25(6):445-449. doi: 10.1097/PAI.0000000000000321. PMID: 26808132
Clinicopathologic and prognostic factors of thymoma in Tunisia.
Braham E, Kilani T, El Mezni F
Tunis Med 2016 Feb;94(2):118-22. PMID: 27532527
Glut-1 intensity and pattern of expression in thymic epithelial tumors are predictive of WHO subtypes.
Thomas de Montpréville V, Quilhot P, Chalabreysse L, De Muret A, Hofman V, Lantuéjoul S, Parrens M, Payan MJ, Rouquette I, Secq V, Girard N, Besse B, Marx A, Molina TJ
Pathol Res Pract 2015 Dec;211(12):996-1002. Epub 2015 Oct 19 doi: 10.1016/j.prp.2015.10.005. PMID: 26534878
Metaplastic thymoma of the mediastinum. A clinicopathologic, immunohistochemical, and genetic analysis.
Liu B, Rao Q, Zhu Y, Yu B, Zhu HY, Zhou XJ
Am J Clin Pathol 2012 Feb;137(2):261-9. doi: 10.1309/AJCP0T1JFYLMPHMI. PMID: 22261452

Diagnosis

A Rare Case of Metaplastic Thymoma Presenting With Myasthenia Gravis.
Vega Prado I, Shymansky J, Apte A, Mortman K, Kaminski HJ, Barak S
Int J Surg Pathol 2024 Feb;32(1):155-159. Epub 2023 Apr 24 doi: 10.1177/10668969231168344. PMID: 37093756
Loss of YAP1 C-terminus expression as an ancillary marker for metaplastic thymoma: a potential pitfall in detecting YAP1::MAML2 gene rearrangement.
Wang X, Liu LL, Li Q, Xia QY, Li R, Ye SB, Zhang RS, Fang R, Chen H, Wu N, Rao Q
Histopathology 2023 Nov;83(5):798-809. Epub 2023 Aug 11 doi: 10.1111/his.15024. PMID: 37565303
Significance of YAP1-MAML2 rearrangement and GTF2I mutation in the diagnosis and differential diagnosis of metaplastic thymoma.
Wang M, Xu H, Han Q, Wang L
Ann Med 2023;55(2):2237040. doi: 10.1080/07853890.2023.2237040. PMID: 37489594Free PMC Article
Malignant Transformation of Metaplastic Thymoma into High-Grade Sarcomatoid Carcinoma: A Case Report.
Piao ZH, Chen JP, Chen HR, Zhou XC
Int J Surg Pathol 2022 Aug;30(5):564-568. Epub 2021 Dec 31 doi: 10.1177/10668969211070484. PMID: 34970930
Metaplastic thymoma of the mediastinum. A clinicopathologic, immunohistochemical, and genetic analysis.
Liu B, Rao Q, Zhu Y, Yu B, Zhu HY, Zhou XJ
Am J Clin Pathol 2012 Feb;137(2):261-9. doi: 10.1309/AJCP0T1JFYLMPHMI. PMID: 22261452

Therapy

Metaplastic thymoma with myasthenia gravis presumably caused by an accumulation of intratumoral immature T cells: a case report.
Tajima S, Yanagiya M, Sato M, Nakajima J, Fukayama M
Int J Clin Exp Pathol 2015;8(11):15375-80. Epub 2015 Nov 1 PMID: 26823897Free PMC Article

Prognosis

Programmed death-ligand 1 expression profiling in thymic epithelial cell tumors: Clinicopathological features and quantitative digital image analyses.
Ishihara S, Okada S, Ogi H, Kodama Y, Shimomura M, Tsunezuka H, Itoh K, Marx A, Inoue M
Lung Cancer 2020 Jul;145:40-47. Epub 2020 May 6 doi: 10.1016/j.lungcan.2020.04.038. PMID: 32402921
Metaplastic thymoma: a distinctive thymic neoplasm characterized by YAP1-MAML2 gene fusions.
Vivero M, Davineni P, Nardi V, Chan JKC, Sholl LM
Mod Pathol 2020 Apr;33(4):560-565. Epub 2019 Oct 22 doi: 10.1038/s41379-019-0382-x. PMID: 31641231
Clinicopathologic and prognostic factors of thymoma in Tunisia.
Braham E, Kilani T, El Mezni F
Tunis Med 2016 Feb;94(2):118-22. PMID: 27532527
Glut-1 intensity and pattern of expression in thymic epithelial tumors are predictive of WHO subtypes.
Thomas de Montpréville V, Quilhot P, Chalabreysse L, De Muret A, Hofman V, Lantuéjoul S, Parrens M, Payan MJ, Rouquette I, Secq V, Girard N, Besse B, Marx A, Molina TJ
Pathol Res Pract 2015 Dec;211(12):996-1002. Epub 2015 Oct 19 doi: 10.1016/j.prp.2015.10.005. PMID: 26534878
Metaplastic thymoma of the mediastinum. A clinicopathologic, immunohistochemical, and genetic analysis.
Liu B, Rao Q, Zhu Y, Yu B, Zhu HY, Zhou XJ
Am J Clin Pathol 2012 Feb;137(2):261-9. doi: 10.1309/AJCP0T1JFYLMPHMI. PMID: 22261452

Clinical prediction guides

Loss of YAP1 C-terminus expression as an ancillary marker for metaplastic thymoma: a potential pitfall in detecting YAP1::MAML2 gene rearrangement.
Wang X, Liu LL, Li Q, Xia QY, Li R, Ye SB, Zhang RS, Fang R, Chen H, Wu N, Rao Q
Histopathology 2023 Nov;83(5):798-809. Epub 2023 Aug 11 doi: 10.1111/his.15024. PMID: 37565303
Programmed death-ligand 1 expression profiling in thymic epithelial cell tumors: Clinicopathological features and quantitative digital image analyses.
Ishihara S, Okada S, Ogi H, Kodama Y, Shimomura M, Tsunezuka H, Itoh K, Marx A, Inoue M
Lung Cancer 2020 Jul;145:40-47. Epub 2020 May 6 doi: 10.1016/j.lungcan.2020.04.038. PMID: 32402921
Clinicopathologic and prognostic factors of thymoma in Tunisia.
Braham E, Kilani T, El Mezni F
Tunis Med 2016 Feb;94(2):118-22. PMID: 27532527
Glut-1 intensity and pattern of expression in thymic epithelial tumors are predictive of WHO subtypes.
Thomas de Montpréville V, Quilhot P, Chalabreysse L, De Muret A, Hofman V, Lantuéjoul S, Parrens M, Payan MJ, Rouquette I, Secq V, Girard N, Besse B, Marx A, Molina TJ
Pathol Res Pract 2015 Dec;211(12):996-1002. Epub 2015 Oct 19 doi: 10.1016/j.prp.2015.10.005. PMID: 26534878

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