Thymic lymphoepitheliomalike carcinoma in children: clinicopathologic features and molecular analysis.
Thymic lymphoepitheliomalike carcinoma (LELC) in children is extremely rare and we report 2 such cases with molecular analysis of Epstein-Barr virus (EBV) and its encoded latent membrane protein-1 as well as a literature review. Both of our patients were male, presented with a huge anterior mediastinal mass, expired within a year after diagnosis despite treatment. There were altogether 9 cases of thymic LELC in children. Their common clinical features include a male predominance, large tumor size, advanced clinical stage, and poor clinical outcome. All cases were associated with EBV and nearly half developed concurrent hypertrophic osteoarthropathy (HOA). Thymic LELC in children is an aggressive EBV-associated malignant tumor frequently accompanied by HOA. We proposed that EBV might play a role in the development of HOA through the interactions of latent membrane protein-1, vascular endothelial growth factor, and cyclooxygenases-2.
Hsueh
Chuen
C
Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Chang Gung Children's Hospital, Tao-Yuan, Taiwan, ROC. ch9211@.adm.cgmh.org.tw
Kuo
Tseng-Tong
TT
Tsang
Ngan-Ming
NM
Wu
Yi-Chin
YC
Yang
Chao-Ping
CP
Hung
Iou-Jih
IJ
D000293:Adolescent; D002277:Carcinoma / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000821:virology; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008479:Mediastinal Neoplasms / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000821:virology; D010004:Osteoarthropathy, Primary Hypertrophic / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000821:virology; D035583:Rare Diseases / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000821:virology; D013953:Thymus Neoplasms / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000821:virology; D014763:Viral Matrix Proteins / Q000235:genetics*
C037662:EBV-associated membrane antigen, Epstein-Barr virus; D014763:Viral Matrix Proteins