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Thymic epithelial neoplasm

MedGen UID:
220416
•Concept ID:
C1266101
•
Neoplastic Process
Synonyms: Epithelial tumor of thymus; Thymic epithelial tumor; Thymoma, adult
SNOMED CT: Thymic epithelial neoplasm (128621008)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
•Concept ID:
C0441748
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Autosomal recessive inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018079
Orphanet: ORPHA3398

Definition

Thymic epithelial neoplasms (TEN) are rare malignancies arising from the epithelium of the thymic gland. They comprise three sub-types: thymoma, thymic carcinoma, and thymic neuroendocrine carcinoma (see these terms). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymic epithelial neoplasm
Follow this link to review classifications for Thymic epithelial neoplasm in Orphanet.

Professional guidelines

PubMed

Chinese expert consensus on the diagnosis and treatment of thymic epithelial tumors.
Xu C, Zhang Y, Wang W, Wang Q, Li Z, Song Z, Wang J, Yu J, Liu J, Zhang S, Cai X, Wu M, Zhan P, Liu H, Lv T, Miao L, Min L, Li J, Liu B, Yuan J, Jiang Z, Lin G, Chen X, Pu X, Rao C, Lv D, Yu Z, Li X, Tang C, Zhou C, Zhang J, Guo H, Chu Q, Meng R, Liu X, Wu J, Hu X, Fang M, Zhou J, Zhu Z, Chen X, Pan W, Pang F, Zhou Y, Jian Q, Wang K, Wang L, Zhu Y, Yang G, Lin X, Cai J, Liang L, Feng H, Wang L, Du Y, Yao W, Shi X, Niu X, Yuan D, Yao Y, Huang J, Zhang Y, Sun P, Wang H, Ye M, Wang D, Wang Z, Hao Y, Wang Z, Wan B, Lv D, Yu G, Li A, Kang J, Zhang J, Zhang C, Chen H, Shi L, Ye L, Wang G, Wang Y, Gao F, Zhou W, Hu C, Wei J, Li B, Li Z, Li Y, Liu Z, Yang N, Wu L, Wang Q, Huang W, Hong Z, Wang G, Fang M, Fang Y, Zhu X, Du K, Ji J, Shen Y, Zhang Y, Ma S, Song Y, Lu Y, Liu A, Fang W, Zhong W
Thorac Cancer 2023 Apr;14(12):1102-1117. Epub 2023 Mar 16 doi: 10.1111/1759-7714.14847. PMID: 36924056Free PMC Article
Surgical, Radiation, and Systemic Treatments of Patients With Thymic Epithelial Tumors: A Clinical Practice Guideline.
Falkson CB, Vella ET, Ellis PM, Maziak DE, Ung YC, Yu E
J Thorac Oncol 2022 Nov;17(11):1258-1275. Epub 2022 Aug 27 doi: 10.1016/j.jtho.2022.08.007. PMID: 36031176
Lung and thymic carcinoids: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Baudin E, Caplin M, Garcia-Carbonero R, Fazio N, Ferolla P, Filosso PL, Frilling A, de Herder WW, Hörsch D, Knigge U, Korse CM, Lim E, Lombard-Bohas C, Pavel M, Scoazec JY, Sundin A, Berruti A; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2021 Apr;32(4):439-451. Epub 2021 Jan 19 doi: 10.1016/j.annonc.2021.01.003. PMID: 33482246

Recent clinical studies

Etiology

Concurrent myasthenia gravis-related cervical thymoma in a patient with diffuse large B-cell lymphoma: a case report.
Kunacheewa C, Wongwiangjunt S, Sukpanichnant S
J Med Case Rep 2022 Jan 12;16(1):10. doi: 10.1186/s13256-021-03225-2. PMID: 35016720Free PMC Article
Diagnostic Value of Dual-time-point F-18 FDG PET/CT and Chest CT for the Prediction of Thymic Epithelial Neoplasms.
Shinya T, Tanaka T, Soh J, Matsushita T, Sato S, Toyooka S, Yoshino T, Miyoshi S, Kanazawa S
Acta Med Okayama 2017 Apr;71(2):105-112. doi: 10.18926/AMO/54978. PMID: 28420891
Staging of thymic epithelial neoplasms: thymoma and thymic carcinoma.
Weissferdt A, Moran CA
Pathol Res Pract 2015 Jan;211(1):2-11. Epub 2014 Jun 24 doi: 10.1016/j.prp.2014.06.007. PMID: 25441660
Stage III thymic epithelial neoplasms are not homogeneous with regard to clinical, pathological, and prognostic features.
Kang MW, Lee ES, Jo J, Han J, Ahn YC, Kim HK, Choi YS, Kim K, Shim YM, Kim J
J Thorac Oncol 2009 Dec;4(12):1561-7. doi: 10.1097/JTO.0b013e3181b9cd7f. PMID: 19745769
Diagnostic utility of thymic epithelial markers CD205 (DEC205) and Foxn1 in thymic epithelial neoplasms.
Nonaka D, Henley JD, Chiriboga L, Yee H
Am J Surg Pathol 2007 Jul;31(7):1038-44. doi: 10.1097/PAS.0b013e31802b4917. PMID: 17592270

Diagnosis

Imaging of the Anterior/Prevascular Mediastinum.
Garrana SH, Rosado-de-Christenson ML
Radiol Clin North Am 2021 Mar;59(2):155-168. Epub 2021 Jan 11 doi: 10.1016/j.rcl.2020.10.003. PMID: 33551078
State of the Art: MR Imaging of Thymoma.
Carter BW, Benveniste MF, Truong MT, Marom EM
Magn Reson Imaging Clin N Am 2015 May;23(2):165-77. doi: 10.1016/j.mric.2015.01.005. PMID: 25952513
Staging of thymic epithelial neoplasms: thymoma and thymic carcinoma.
Weissferdt A, Moran CA
Pathol Res Pract 2015 Jan;211(1):2-11. Epub 2014 Jun 24 doi: 10.1016/j.prp.2014.06.007. PMID: 25441660
Diagnostic utility of thymic epithelial markers CD205 (DEC205) and Foxn1 in thymic epithelial neoplasms.
Nonaka D, Henley JD, Chiriboga L, Yee H
Am J Surg Pathol 2007 Jul;31(7):1038-44. doi: 10.1097/PAS.0b013e31802b4917. PMID: 17592270
Diagnosis of thymoma.
Suster S
J Clin Pathol 2006 Dec;59(12):1238-44. Epub 2006 May 5 doi: 10.1136/jcp.2005.031138. PMID: 16679354Free PMC Article

Prognosis

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
Reproducibility of 3 histologic classifications and 3 staging systems for thymic epithelial neoplasms and its effect on prognosis.
Roden AC, Yi ES, Jenkins SM, Edwards KK, Donovan JL, Lewis JE, Cassivi SD, Marks RS, Garces YI, Aubry MC
Am J Surg Pathol 2015 Apr;39(4):427-41. doi: 10.1097/PAS.0000000000000391. PMID: 25634747
Staging of thymic epithelial neoplasms: thymoma and thymic carcinoma.
Weissferdt A, Moran CA
Pathol Res Pract 2015 Jan;211(1):2-11. Epub 2014 Jun 24 doi: 10.1016/j.prp.2014.06.007. PMID: 25441660
Stage III thymic epithelial neoplasms are not homogeneous with regard to clinical, pathological, and prognostic features.
Kang MW, Lee ES, Jo J, Han J, Ahn YC, Kim HK, Choi YS, Kim K, Shim YM, Kim J
J Thorac Oncol 2009 Dec;4(12):1561-7. doi: 10.1097/JTO.0b013e3181b9cd7f. PMID: 19745769
Diagnosis of thymoma.
Suster S
J Clin Pathol 2006 Dec;59(12):1238-44. Epub 2006 May 5 doi: 10.1136/jcp.2005.031138. PMID: 16679354Free PMC Article

Clinical prediction guides

Novel Tumor-Specific Antigens for Immunotherapy Identified From Multi-omics Profiling in Thymic Carcinomas.
Fang W, Wu CH, Sun QL, Gu ZT, Zhu L, Mao T, Zhang XF, Xu N, Lu TP, Tsai MH, Chen LH, Lai LC, Chuang EY
Front Immunol 2021;12:748820. Epub 2021 Nov 16 doi: 10.3389/fimmu.2021.748820. PMID: 34867976Free PMC Article
Diagnostic Value of Dual-time-point F-18 FDG PET/CT and Chest CT for the Prediction of Thymic Epithelial Neoplasms.
Shinya T, Tanaka T, Soh J, Matsushita T, Sato S, Toyooka S, Yoshino T, Miyoshi S, Kanazawa S
Acta Med Okayama 2017 Apr;71(2):105-112. doi: 10.18926/AMO/54978. PMID: 28420891
Reproducibility of 3 histologic classifications and 3 staging systems for thymic epithelial neoplasms and its effect on prognosis.
Roden AC, Yi ES, Jenkins SM, Edwards KK, Donovan JL, Lewis JE, Cassivi SD, Marks RS, Garces YI, Aubry MC
Am J Surg Pathol 2015 Apr;39(4):427-41. doi: 10.1097/PAS.0000000000000391. PMID: 25634747
Diagnosis of thymoma.
Suster S
J Clin Pathol 2006 Dec;59(12):1238-44. Epub 2006 May 5 doi: 10.1136/jcp.2005.031138. PMID: 16679354Free PMC Article
Spindle cell thymic carcinoma: clinicopathologic and immunohistochemical study of a distinctive variant of primary thymic epithelial neoplasm.
Suster S, Moran CA
Am J Surg Pathol 1999 Jun;23(6):691-700. doi: 10.1097/00000478-199906000-00009. PMID: 10366152

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