A case of split notochord syndrome: An adult with a spinal endodermal cyst mimicking an intramedullary tumor
A case of split notochord syndrome: An adult with a spinal endodermal cyst mimicking an intramedullary tumor
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DOI：
10.1111/j.1440-1789.2011.01212.x
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发表时间：
2011-12-01
期刊：
影响因子：
2.3
通讯作者：
Ishiguro, Naoki
中科院分区：
文献类型：
--
作者：
Wakao, Norimitsu;Imagama, Shiro;Ishiguro, Naoki
椎管内内胚层囊肿是非常罕见的先天性囊肿，通常由薄壁囊肿组成，其衬里类似胃肠道或呼吸道上皮。诊断通常在生命的第一个或第二个十年确定。这些囊肿通常与椎体或脊髓异常以及纵膈或腹腔囊肿的双重畸形有关。它们统称为脊索分裂综合征。作者描述了他们的经验，在治疗一个57岁的男人有一个内胚层囊肿模拟髓内肿瘤在Th1-2水平。他在经历短暂的左下肢麻木和无力后，在当地一家医院通过MRI诊断出椎管内肿块，并被送入我们的机构进行评估。t1加权矢状面MRI显示病灶的信号强度为脊髓等至微低，钆治疗后无增强。虽然t2加权矢状位图像显示脊髓高信号，但轴向图像显示肿块和蛛网膜下腔之间有通道。我们不能完全排除髓内肿瘤的存在，并采用后路椎板切除术。组织病理学分析显示为内胚层囊肿，作者发现脊髓空洞，术前矢状位MRI和术中超声检查与囊肿明显分离。据我们所知，这是英文文献中第一例需要与脊髓肿瘤鉴别诊断的胸部内胚层囊肿。
Intraspinal endodermal cysts are very rare congenital cysts, usually composed of a thin-walled cyst the lining of which mimics gastrointestinal or respiratory epithelium. Diagnosis is usually established during the first or second decade of life. These cysts are frequently associated with vertebral or spinal cord abnormalies and dual malformation with mediastinal or abdominal cysts. Collectively, they are called split notochord syndrome. The authors describe their experience in the treatment of a 57-year-old man having an endodermal cyst mimicking an intramedullary tumor at the level of Th1-2. He was admitted to our institution for evaluation of an intraspinal mass diagnosed by MRI at a local hospital after experiencing temporary numbness and weakness of the lower left extremity. T1-weighted sagittal MRI demonstrated the lesion with signal intensity iso- to slightly hypointense to the spinal cord without enhancement after administration of gadolinium. Although T2-weighted sagittal images demonstrated as hyperintense to the spinal cord, axial images revealed a passage between the mass and subarachnoid space. We could not completely rule out the presence of an intramedullary tumor and undertook a laminectomy with a posterior approach. Histopathological analysis revealed an endodermal cyst and the authors found syringomyelia, which was clearly separated from the cyst in the preoperative sagittal MRI and intraoperative ultrasonography study. To the best of our knowledge, this is the first report in the English literature of a thoracic endodermal cyst requiring differential diagnosis from a spinal cord tumor.