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Thymoma type AB

MedGen UID:
220414
•Concept ID:
C1266092
•
Neoplastic Process
Synonyms: Mixed Type Thymoma; mixed type thymoma; Primary thymic epithelial neoplasm type AB; primary thymic epithelial neoplasm type AB; primary thymic epithelial tumor type AB; Primary thymic epithelial tumor type AB; primary thymic epithelial tumour type AB; Thymoma Type AB; thymoma type AB; thymoma, mixed type
SNOMED CT: Thymoma type AB (1144922006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016975
Orphanet: ORPHA263324

Definition

A thymic epithelial neoplasm characterized by the presence of a lymphocyte-poor component similar to that seen in type A thymoma and a lymphocyte-rich component which contains neoplastic small polygonal epithelial cells. It may be associated with myasthenia gravis and pure red cell aplasia. The majority of cases occur in the anterior mediastinum as Masaoka stage I tumors. A minority of the cases occur as stage II or stage III tumors. The overall survival is reported to be 80-100% at 5 and 10 years. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymoma type AB
Follow this link to review classifications for Thymoma type AB in Orphanet.

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

Associated immunological disorders and cellular immune dysfunction in thymoma: a study of 87 cases from Thailand.
Thongprayoon C, Tantrachoti P, Phatharacharukul P, Buranapraditkun S, Klaewsongkram J
Arch Immunol Ther Exp (Warsz) 2013 Feb;61(1):85-93. Epub 2012 Dec 11 doi: 10.1007/s00005-012-0207-9. PMID: 23229679
Recurrent myasthenia gravis due to a pleural implant 3 years after radical thymectomy.
Heyman SR, De Raeve H, Mercelis R, De Pooter C, Van Schil P
Ann Thorac Surg 2008 Jul;86(1):299-301. doi: 10.1016/j.athoracsur.2008.01.030. PMID: 18573446

Diagnosis

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
Ectopic thymoma in the middle mediastinum: A case report and literature review.
Tuan PA, Minh Duc N
Clin Ter 2021 Mar 15;172(2):94-98. doi: 10.7417/CT.2021.2291. PMID: 33763676
Concurrent thymoma, thymic carcinoma, and T lymphoblastic leukemia/lymphoma in an anterior mediastinal mass.
Ito J, Yoshida A, Maeshima AM, Nakagawa K, Watanabe S, Kobayashi Y, Fukuhara S, Tsuta K
Pathol Res Pract 2015 Sep;211(9):693-6. Epub 2015 Jun 9 doi: 10.1016/j.prp.2015.06.002. PMID: 26150396
Associated immunological disorders and cellular immune dysfunction in thymoma: a study of 87 cases from Thailand.
Thongprayoon C, Tantrachoti P, Phatharacharukul P, Buranapraditkun S, Klaewsongkram J
Arch Immunol Ther Exp (Warsz) 2013 Feb;61(1):85-93. Epub 2012 Dec 11 doi: 10.1007/s00005-012-0207-9. PMID: 23229679
18F-fluorodeoxyglucose and 11C-acetate positron emission tomography are useful modalities for diagnosing the histologic type of thymoma.
Shibata H, Nomori H, Uno K, Sakaguchi K, Nakashima R, Iyama K, Tomiyoshi K, Kaji M, Goya T, Suzuki T, Horio H
Cancer 2009 Jun 1;115(11):2531-8. doi: 10.1002/cncr.24278. PMID: 19326456

Therapy

Seronegative panencephalitis complicated by viral encephalomyelitis in a case of Good's syndrome - a neuropathological report.
Ray S, Kathuria H, Chakravarty K, Rawat A, Takkar A, Mehta S, Goyal M, Ahuja C, Bhatia V, Lal V, Gupta K
Int J Neurosci 2022 Oct;132(10):1031-1036. Epub 2020 Dec 15 doi: 10.1080/00207454.2020.1860040. PMID: 33280465
Associated immunological disorders and cellular immune dysfunction in thymoma: a study of 87 cases from Thailand.
Thongprayoon C, Tantrachoti P, Phatharacharukul P, Buranapraditkun S, Klaewsongkram J
Arch Immunol Ther Exp (Warsz) 2013 Feb;61(1):85-93. Epub 2012 Dec 11 doi: 10.1007/s00005-012-0207-9. PMID: 23229679
18F-fluorodeoxyglucose and 11C-acetate positron emission tomography are useful modalities for diagnosing the histologic type of thymoma.
Shibata H, Nomori H, Uno K, Sakaguchi K, Nakashima R, Iyama K, Tomiyoshi K, Kaji M, Goya T, Suzuki T, Horio H
Cancer 2009 Jun 1;115(11):2531-8. doi: 10.1002/cncr.24278. PMID: 19326456
Recurrent myasthenia gravis due to a pleural implant 3 years after radical thymectomy.
Heyman SR, De Raeve H, Mercelis R, De Pooter C, Van Schil P
Ann Thorac Surg 2008 Jul;86(1):299-301. doi: 10.1016/j.athoracsur.2008.01.030. PMID: 18573446

Prognosis

18F-fluorodeoxyglucose and 11C-acetate positron emission tomography are useful modalities for diagnosing the histologic type of thymoma.
Shibata H, Nomori H, Uno K, Sakaguchi K, Nakashima R, Iyama K, Tomiyoshi K, Kaji M, Goya T, Suzuki T, Horio H
Cancer 2009 Jun 1;115(11):2531-8. doi: 10.1002/cncr.24278. PMID: 19326456
Recurrent myasthenia gravis due to a pleural implant 3 years after radical thymectomy.
Heyman SR, De Raeve H, Mercelis R, De Pooter C, Van Schil P
Ann Thorac Surg 2008 Jul;86(1):299-301. doi: 10.1016/j.athoracsur.2008.01.030. PMID: 18573446

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
Ectopic thymoma in the middle mediastinum: A case report and literature review.
Tuan PA, Minh Duc N
Clin Ter 2021 Mar 15;172(2):94-98. doi: 10.7417/CT.2021.2291. PMID: 33763676
18F-fluorodeoxyglucose and 11C-acetate positron emission tomography are useful modalities for diagnosing the histologic type of thymoma.
Shibata H, Nomori H, Uno K, Sakaguchi K, Nakashima R, Iyama K, Tomiyoshi K, Kaji M, Goya T, Suzuki T, Horio H
Cancer 2009 Jun 1;115(11):2531-8. doi: 10.1002/cncr.24278. PMID: 19326456

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