Song Xianyuan, Choi Jai, Rao Chandrakant, Nallu Sagarika, Nicastri Anthony D
Department of Pathology, SUNY Downstate Medical Center, Brooklyn, New York 11203, USA.
Pediatr Neurol. 2008 Jan;38(1):58-60. doi: 10.1016/j.pediatrneurol.2007.09.003.
Primary vertebral Ewing sarcoma-primitive neuroectodermal tumor is uncommon. Although epidural extension has been seen in such tumors, cases with massive intraspinal involvement are decidedly rare. Here we present the case of a 4-year-old girl with back pain and difficulty walking. Magnetic resonance imaging showed a mass filling the spinal canal from T(11) to the L(3)/L(4) levels. Vertebral involvement with extension into the paraspinal soft tissue through neural foramina was seen. Histologically, a small-blue-cell tumor with strong membranous CD99 reactivity was noted. Molecular analysis revealed translocation t(11;22)(q24;q12), thus confirming the diagnosis of Ewing sarcoma-primitive neuroectodermal tumor. Our case emphasizes that vertebral Ewing sarcoma-primitive neuroectodermal tumor may present with massive intraspinal extension and should be included in the differential diagnosis of intraspinal lesions.
原发性脊柱尤文肉瘤-原始神经外胚层肿瘤并不常见。虽然此类肿瘤可见硬膜外扩展，但脊髓内大量受累的病例确实罕见。在此，我们报告一例4岁女童，有背痛及行走困难症状。磁共振成像显示一个肿块占据了从胸11至腰3/腰4水平的椎管。可见椎体受累并通过神经孔延伸至椎旁软组织。组织学检查发现一个小蓝细胞肿瘤，CD99膜反应性强。分子分析显示t(11;22)(q24;q12)易位，从而确诊为尤文肉瘤-原始神经外胚层肿瘤。我们的病例强调，原发性脊柱尤文肉瘤-原始神经外胚层肿瘤可能表现为脊髓内大量扩展，应列入脊髓内病变的鉴别诊断。
