Epstein-Barr virus-associated thymic lymphoepithelial carcinoma in a child.

Abstract

Thymic lymphoepithelial carcinoma is a rare neoplasm in children, with histological features similar to those of nasopharyngeal carcinoma and a frequent association with the Epstein-Barr virus (EBV). Diagnosis is challenging due to clinical and imaging overlaps with lymphomas and mediastinal germ cell tumors. We present the case of a 12-year-old patient with a mediastinal mass, supraclavicular lymphadenopathy, and pulmonary metastases, histologically diagnosed as EBV-positive thymic lymphoepithelial carcinoma. He received chemotherapy with cisplatin and gemcitabine, immunotherapy with pembrolizumab, and radiation therapy, and achieved complete remission. The literature reports few pediatric cases, with a poor prognosis and a median survival of less than 24 months. However, the addition of anti-PD-1 immunotherapy in EBV-positive tumors appears to offer clinical benefit. This case highlights the importance of a multidisciplinary approach and the potential benefits of combination therapies, including immunotherapy, in EBV-positive mediastinal tumors in children.