Section of Neurosurgery, Department of Surgery, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Department of Diagnostic Radiology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
World Neurosurg. 2020 Feb;134:489-494. doi: 10.1016/j.wneu.2019.11.069. Epub 2019 Nov 19.
Neurenteric cyst (NEC) is a rare intradural spinal tumor, but a correct preoperative diagnosis remains challenging. A misdiagnosis of arachnoid cyst (AC) often leads to conflicting surgical management and significantly higher recurrence.
We report the case of a 26-year-old woman who presented with progressive spastic quadriparesis with myelopathy below the C4 level, which was caused by a ventral intradural extramedullary cystic tumor at the C3-4 level. Magnetic resonance images showed the cystic content as identical to cerebrospinal fluid, which prompted the tentative diagnosis of spinal AC. Surgical fenestration was scheduled. However, intraoperative findings of a thick-walled cyst and severe adhesion to the neural structure without a history of trauma and inflammation were more compatible with the pathogenesis of an NEC. Because of the high recurrence rate after an incomplete resection of an NEC, we did a complete resection of the cyst with adhesive rootlets instead. Pathology analysis and immunohistochemical staining confirmed the diagnosis of an endodermal-derived NEC.
NECs must be differentiated from ACs because they are different diseases and require different surgical management. In cases with clear cystic content, however, the diagnosis is likely to be AC, but a thick cystic wall and structural adhesions should suggest the differential diagnosis of NEC. Gross total removal of NECs should be attempted to reduce NEC recurrence.
神经肠上皮囊肿(NEC)是一种罕见的硬脊膜内脊髓肿瘤,但正确的术前诊断仍然具有挑战性。蛛网膜囊肿(AC)的误诊常导致手术管理冲突,并显著增加复发率。
我们报告了一例 26 岁女性病例,其因 C3-4 水平的腹侧硬脊膜外髓外囊性肿瘤导致 C4 以下水平进行性痉挛性四肢瘫痪和脊髓病。磁共振成像显示囊性内容与脑脊液相同,提示脊髓 AC 的初步诊断。计划进行手术开窗。然而,术中发现厚壁囊肿,并与神经结构严重粘连,无创伤和炎症史,更符合 NEC 的发病机制。由于 NEC 不完全切除后复发率高,我们对囊肿及其粘连的神经根进行了完全切除。病理分析和免疫组织化学染色证实了内胚层源性 NEC 的诊断。
必须将 NEC 与 AC 区分开来,因为它们是两种不同的疾病,需要不同的手术治疗。然而,在囊性内容明确的情况下,诊断可能是 AC,但囊性壁增厚和结构粘连应提示 NEC 的鉴别诊断。应尝试完全切除 NEC 以降低 NEC 复发率。