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Lymphoepithelioma-like thymic carcinoma

MedGen UID:
79026
•Concept ID:
C0279706
•
Neoplastic Process
Synonyms: Lymphoepithelial Cancer of the Thymus; lymphoepithelial cancer of the Thymus; Lymphoepithelial Cancer of Thymus; lymphoepithelial cancer of Thymus; Lymphoepithelial Thymus Cancer; lymphoepithelial Thymus cancer; Lymphoepithelioma-like Carcinoma of the Thymus; lymphoepithelioma-like carcinoma of the Thymus; Lymphoepithelioma-like Carcinoma of Thymus; lymphoepithelioma-like carcinoma of Thymus; Lymphoepithelioma-like Thymic Carcinoma; lymphoepithelioma-like thymic carcinoma; Lymphoepithelioma-like Thymus Carcinoma; lymphoepithelioma-like Thymus carcinoma; Malignant Lymphoepithelial Thymoma; malignant lymphoepithelial thymoma; Thymic Lymphoepithelial Carcinoma; Thymic Lymphoepithelioma-Like Carcinoma; thymic lymphoepithelioma-like carcinoma
 
Monarch Initiative: MONDO:0004297

Definition

A rare, usually aggressive primary thymic carcinoma, characterized by a syncytial growth of undifferentiated carcinoma cells and the presence of a lymphoplasmacytic infiltrate. More than 40% of cases are associated with Epstein-Barr virus infection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphoepithelioma-like thymic carcinoma

Professional guidelines

PubMed

About thymic carcinomas: challenges in diagnosis and management.
Mlika M, Boudaya S, Braham E, Chermiti F, Sayi A, Marghli A, El Mezni F
Asian Cardiovasc Thorac Ann 2016 May;24(4):350-4. Epub 2016 Mar 21 doi: 10.1177/0218492316640129. PMID: 27002097
Correlation between S-1 treatment outcome and expression of biomarkers for refractory thymic carcinoma.
Okuma Y, Hosomi Y, Miyamoto S, Shibuya M, Okamura T, Hishima T
BMC Cancer 2016 Feb 25;16:156. doi: 10.1186/s12885-016-2159-7. PMID: 26915359Free PMC Article
Thymic carcinoma. Outcome of treatment including surgical resection.
Takahashi K, Yoshida J, Nishimura M, Nagai K
Jpn J Thorac Cardiovasc Surg 2000 Aug;48(8):494-8. doi: 10.1007/BF03218184. PMID: 11002578

Recent clinical studies

Diagnosis

Poorly Differentiated Nonkeratinizing Squamous Cell Carcinoma of the Thymus: Clinicopathologic and Molecular Genetic Study of 25 Cases.
Suster D, Pihan G, Mackinnon AC, Suster S
Am J Surg Pathol 2018 Sep;42(9):1224-1236. doi: 10.1097/PAS.0000000000001101. PMID: 29975245
A rare highly aggressive tumour: lymphoepithelioma-like thymic carcinoma.
Gomez JMD, Syed G, Co MLF, Bayoumi M, Abrams R
BMJ Case Rep 2017 Sep 23;2017 doi: 10.1136/bcr-2017-221478. PMID: 28942409Free PMC Article
Multiple brain metastases from "lymphoepithelioma-like" thymic carcinoma: a combined stereotactic-radiosurgical approach.
Nicolato A, Ferraresi P, Bontempini L, Tomazzoli L, Magarotto R, Gerosa M
Surg Neurol 2001 Apr;55(4):232-4. doi: 10.1016/s0090-3019(01)00361-5. PMID: 11358599

Therapy

Lymphoepithelioma-like thymic carcinoma in a 16-year-old boy with nephrotic syndrome--a case report.
Kiliś-Pstrusińska K, Medyńska A, Zwolińska D, Dobaczewski G
Pediatr Nephrol 2008 Jun;23(6):1001-3. doi: 10.1007/s00467-007-0666-0. PMID: 18046582

Prognosis

Poorly Differentiated Nonkeratinizing Squamous Cell Carcinoma of the Thymus: Clinicopathologic and Molecular Genetic Study of 25 Cases.
Suster D, Pihan G, Mackinnon AC, Suster S
Am J Surg Pathol 2018 Sep;42(9):1224-1236. doi: 10.1097/PAS.0000000000001101. PMID: 29975245
A rare highly aggressive tumour: lymphoepithelioma-like thymic carcinoma.
Gomez JMD, Syed G, Co MLF, Bayoumi M, Abrams R
BMJ Case Rep 2017 Sep 23;2017 doi: 10.1136/bcr-2017-221478. PMID: 28942409Free PMC Article
Lymphoepithelioma-like thymic carcinoma in a 16-year-old boy with nephrotic syndrome--a case report.
Kiliś-Pstrusińska K, Medyńska A, Zwolińska D, Dobaczewski G
Pediatr Nephrol 2008 Jun;23(6):1001-3. doi: 10.1007/s00467-007-0666-0. PMID: 18046582
Thymic carcinoma in children.
Yaris N, Nas Y, Cobanoglu U, Yavuz MN
Pediatr Blood Cancer 2006 Aug;47(2):224-7. doi: 10.1002/pbc.20468. PMID: 16007580
Squamous carcinoma of the thymus.
Chen KT
J Surg Oncol 1984 Jan;25(1):61-3. doi: 10.1002/jso.2930250117. PMID: 6694395

Clinical prediction guides

Poorly Differentiated Nonkeratinizing Squamous Cell Carcinoma of the Thymus: Clinicopathologic and Molecular Genetic Study of 25 Cases.
Suster D, Pihan G, Mackinnon AC, Suster S
Am J Surg Pathol 2018 Sep;42(9):1224-1236. doi: 10.1097/PAS.0000000000001101. PMID: 29975245
Epstein-Barr virus (EBV) associated lymphoepithelioma-like thymic carcinoma associated with paraneoplastic syndrome of polymyositis: a rare tumor with rare association.
Koppula BR, Pipavath S, Lewis DH
Clin Nucl Med 2009 Oct;34(10):686-8. doi: 10.1097/RLU.0b013e3181b53f5a. PMID: 19893401
Squamous carcinoma of the thymus.
Chen KT
J Surg Oncol 1984 Jan;25(1):61-3. doi: 10.1002/jso.2930250117. PMID: 6694395

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