Thymomas in childhood: 11 cases from a single institution.

ABSTRACT

AIM: Thymomas are rare in children. Our aim was to review clinical and pathologic characteristics and outcome in children with thymomas. PATIENTS AND METHODS: Between 1979 and 2011, 11 children with thymomas were diagnosed. Hospital files were reviewed for presenting complaints, clinical, radiologic, and other laboratory data, surgical practices, chemotherapy and radiotherapy outcomes. RESULTS: Median age was 8 years (range, 1 to 13 y). Male to female ratio was 9/2. Most common initial complaints were dyspnea, cough, chest pain, and fever. Median time from onset of symptoms was 1 month (range, 0.23 to 3 mo). Associated conditions including hyper-IgE syndrome, hypogammaglobulinemia, and systemic lupus erythematosus plus idiopathic thrombocytopenic purpura were present in 3 patients. Chest x-rays and/or thoracic computed tomographies displayed thymic hyperplasia and/or masses in anterior mediastinum accompanied by pleural (n = 2) and pericardial effusions (n = 1), pulmonary metastases (n = 1), and cervical lymph node metastasis (n = 1). Compression or invasion of trachea or vessels was documented in 5 cases. Seven cases underwent initial tumor resection; others experienced open or trucut biopsies. Histopathologically, 5 cases had invasive and 6 had benign thymomas. Benign thymomas did not receive any postoperative treatment; all cases are disease free at a median follow-up of 211 months. Three of 5 cases with invasive thymomas underwent surgery, 4/5 received chemotherapy and external radiotherapy (3600 to 4500 cGy). Two invasive thymomas died of disease. Three cases with invasive thymomas are disease free at a median follow-up of 209 months. CONCLUSIONS: Benign thymomas have excellent prognosis. For invasive thymomas with or without metastasis, radiotherapy, and chemotherapy offers survival advantage. Complete surgical resection may increase chances for cure.