Stark Andreas M, Leuschner Ivo, Mehdorn H Maximilian, Claviez Alexander
Department of Neurosurgery, Schleswig-Holstein Medical University in Kiel, Arnold-Heller Strasse 3, Haus 41, 24105 Kiel, Germany.
Institute of Pathology, Children's Tumor Registry, Schleswig-Holstein Medical University in Kiel, Arnold-Heller Strasse 3, Haus 14, 24105 Kiel, Germany.
Case Rep Med. 2014;2014:439830. doi: 10.1155/2014/439830. Epub 2014 Dec 29.
Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearranged EWSR-1 gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.
髓母细胞瘤、星形细胞瘤和室管膜瘤是儿童期最常见的幕下肿瘤，而该部位的尤因肉瘤极为罕见。一名12岁女孩被诊断出左侧幕下有一个较大的占位性病变，伴有颅内压升高的症状。肿瘤全切术后，组织学和分子检查显示为尤因肉瘤，EWSR-1基因重排。根据欧洲尤因肉瘤2008治疗方案，患者在接受辅助化疗和放疗后实现了完全缓解。颅内尤因肉瘤虽然罕见，但应作为儿童颅内肿瘤的重要鉴别诊断，需要积极的多模式治疗。
