Niehues T, Harms D, Jürgens H, Göbel U
Departments of Pediatric Hematology and Oncology, University of Düsseldorf, Germany.
Med Pediatr Oncol. 1996 Jun;26(6):419-24. doi: 10.1002/(SICI)1096-911X(199606)26:6<419::AID-MPO10>3.0.CO;2-6.
Malignant thymoma, including thymic carcinoma, is extremely uncommon in the pediatric population. It is known to have a very poor outcome. We report on a 14-year-old boy with Epstein-Barr virus (EBV)-associated thymic carcinoma. Sections of the original tumor were analyzed for EBV by in situ hybridization to confirm the histological diagnosis of a lymphoepithelioma-like subtype. High copy numbers of EBV RNA were detected in the tumor tissue, suggesting an etiological role of EBV in our case. Intensive treatment resulted in long-term remission over 12 years. In order to facilitate the difficult management of the rare child with malignant thymoma, a literature search was initiated. Forty well-documented pediatric cases of malignant thymoma were found in the literature. Histological characteristics, clinical features, and therapeutic regimens were reviewed. Having the very limited experience with malignant thymoma in childhood in mind, it is concluded that its aggressiveness makes the most intensive treatment necessary. Long-term remission can be achieved by application of radical surgery, high-dose irradiation, and multiagent chemotherapy. The combination of cisplatinum, etoposide, and ifosfamide seems to be promising.
恶性胸腺瘤，包括胸腺癌，在儿科人群中极为罕见。已知其预后非常差。我们报告了一名14岁患爱泼斯坦-巴尔病毒（EBV）相关胸腺癌的男孩。通过原位杂交对原发肿瘤切片进行EBV分析，以确认淋巴上皮瘤样亚型的组织学诊断。在肿瘤组织中检测到高拷贝数的EBV RNA，提示EBV在我们的病例中具有病因学作用。强化治疗导致12年以上的长期缓解。为了便于对罕见的儿童恶性胸腺瘤进行困难的管理，我们开展了文献检索。在文献中发现了40例记录良好的儿童恶性胸腺瘤病例。对其组织学特征、临床特征和治疗方案进行了综述。鉴于儿童恶性胸腺瘤的经验非常有限，得出的结论是其侵袭性使得最强化的治疗成为必要。通过根治性手术、高剂量放疗和多药化疗可以实现长期缓解。顺铂、依托泊苷和异环磷酰胺的联合应用似乎很有前景。
