Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis.
Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis.
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DOI：
10.4103/2156-7514.126050
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发表时间：
2014
影响因子：
0.9
通讯作者：
Abdullaev Z
中科院分区：
文献类型：
--
作者：
Mardekian SK;Gandhe A;Miettinen M;Pack S;Curtis MT;Abdullaev Z
骨外尤文氏肉瘤/外周神经外胚层肿瘤（ES/PNDT）是一种罕见的肿瘤，约占儿童软组织肉瘤的10%-15%，成人软组织肉瘤的5%。原发性脊柱、骨外、硬膜内ES/PNIPs更不常见。ES/PNET的诊断极具挑战性，因为肿瘤可能具有非特异性的放射学表现，并且组织学特征与许多其他“小圆细胞肿瘤”相同。因此，ES/PNET应包括在放射学和病理学鉴别诊断，即使在老年患者和不寻常的肿瘤部位。我们报告了两例脊柱，骨外，硬膜内ES/PNIPs的成年人谁提出的背痛。磁共振成像显示对比增强，在脊髓圆锥区域的硬膜内病变。病例1的肿瘤部分位于髓内，而病例2的肿瘤完全位于髓外。在这两个病例中，影像学和术中手术的印象有利于室管膜瘤。ES/PNET的诊断建立在这两种情况下，通过组织病理学，免疫组化和分子分析。
Extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors (ES/PNETs) are rare neoplasms that account for approximately 10%-15% of soft tissue sarcomas in children and 5% of soft tissue sarcomas in adults. Primary spinal, extraosseous, intradural ES/PNETs are even less common. The diagnosis of ES/PNET is extremely challenging, because the tumor can have a nonspecific radiologic appearance, and the histologic features are shared by many other “small round cell tumors.” Thus, ES/PNET should be included in the radiologic and pathologic differential diagnosis, even in older patients and in unusual tumor sites. We report two cases of spinal, extraosseous, intradural ES/PNETs in adults who presented with back pain. Magnetic resonance imaging revealed contrast enhancing, intradural lesions in the area of the conus medullaris. The tumor in Case 1 was partially intramedullary, while the tumor in Case 2 was exclusively extramedullary. In both cases, the radiologic and intraoperative surgical impression favored ependymoma. The diagnosis of ES/PNET was established in both cases by histopathologic, immunohistochemical, and molecular analysis.