Thymic lymphoepitheliomalike carcinoma in children: Clinicopathologic features and molecular analysis

Chuen Hsueh*, Tseng Tong Kuo, Ngan Ming Tsang, Yi Chin Wu, Chao Ping Yang, Iou Jih Hung

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

15 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)785-790
Number of pages6
JournalJournal of Pediatric Hematology/Oncology
Volume28
Issue number12
DOIs
StatePublished - 12 2006

Keywords

  • Children
  • Epstein-Barr virus
  • Hypertrophic osteoarthropathy
  • Latent membrane protein-1
  • Lymphoepitheliomalike carcinoma
  • Thymus

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