Department of Surgery, Duke University Medical Center, Durham, NC, USA.
Neurosurgery. 2011 Oct;69(4):E995-9. doi: 10.1227/NEU.0b013e318223b7c7.
To report a rare case of spinal intradural extraosseous Ewing sarcoma in an adult and review current literature. Although Ewing sarcoma belongs to the family, the treatment modalities are different, and thus the correct diagnosis is very important despite its rare occurrence.
A 56-year-old woman presented with nocturnal bilateral buttock and leg pain. Magnetic resonance imaging (MRI) showed an enhancing intradural extramedullary extraosseous tumor at L1.
A T12-L2 laminectomy was performed to resect the tumor. Immunohistochemical analysis confirmed the diagnosis of Ewing sarcoma. A thorough diagnostic workup did not reveal any bony origin of the tumor. Primary intradural central nervous system Ewing sarcoma is infrequently encountered and shares imaging and histopathological features with central primitive neuroectodermal tumors. Establishment of the right diagnosis is crucial because it mandates a distinct workup and treatment modality different from that for central primitive neuroectodermal tumor. Although osseous Ewing sarcoma predominantly occurs in children and young adults, extraosseous central nervous system Ewing sarcoma is not uncommon in adults and should therefore be considered in the differential diagnosis of extraosseous small blue cell tumors in adult patients.
报告一例成人罕见的脊柱硬膜内骨外尤文肉瘤病例，并复习目前的文献。尽管尤文肉瘤属于家族，但治疗方式不同，因此尽管其罕见，但正确的诊断非常重要。
一名 56 岁女性出现夜间双侧臀部和腿部疼痛。磁共振成像（MRI）显示 L1 处增强的硬膜外髓外骨外肿瘤。
进行 T12-L2 椎板切除术以切除肿瘤。免疫组织化学分析证实了尤文肉瘤的诊断。彻底的诊断性检查未发现肿瘤的任何骨起源。原发性硬脑膜内中枢神经系统尤文肉瘤很少见，其影像学和组织病理学特征与中枢原始神经外胚层肿瘤相似。正确诊断至关重要，因为它需要不同的检查和治疗方式，与中枢原始神经外胚层肿瘤不同。尽管骨尤文肉瘤主要发生在儿童和青少年，但硬脑膜外中枢神经系统尤文肉瘤在成人中并不罕见，因此在成人骨外小蓝细胞肿瘤的鉴别诊断中应考虑到这一点。
