Mittal Sandeep, Petrecca Kevin, Sabbagh Abdulrahman J, Rayes Mahmoud, Melançon Denis, Guiot Marie-Christine, Olivier André
Department of Neurosurgery, Wayne State University, Detroit Medical Center, MI 48201, USA.
Clin Neurol Neurosurg. 2010 Feb;112(2):89-97. doi: 10.1016/j.clineuro.2009.11.001. Epub 2009 Dec 3.
The histopathological, immunologic, and ultrastructural findings of neurenteric cysts support an endodermal derivation. These developmental cystic lesions are generally located in the posterior mediastinum, abdomen, and pelvis and may also contain some mesodermal and neuroectodermal elements. In contrast, neurenteric cysts of the central nervous system are very infrequent and occur most commonly in the spinal canal. Intraspinal neurenteric cysts are usually encountered in the cervicothoracic region with an intradural, extramedullary location and are commonly associated with congenital defects of the overlying skin and/or vertebral bodies. Intracranial neurenteric cysts are very uncommon and typically located in the posterior fossa. Several hypotheses have been postulated to explain the origin of intracranial neurenteric cysts. However, the embryologic basis of these fascinating lesions remains incompletely understood. Supratentorial neurenteric cysts are distinctly rare often represent a diagnostic challenge on preoperative neuroimaging. In fact, only 22 cases of supratentorial neurenteric cysts have been reported in the literature including our own patient with a laterally based convexity extraaxial cyst presenting with seizures. In this report, we review the clinical, radiographic, and histological findings of supratentorial neurenteric cysts. We discuss the differential diagnoses and surgical considerations in the management of these intriguing lesions. We also provide an extensive review of normal human embryogenesis and discuss putative mechanisms of embryopathogenesis of supratentorial neurenteric cysts.
神经肠囊肿的组织病理学、免疫学及超微结构表现支持其来源于内胚层。这些发育性囊性病变通常位于后纵隔、腹部及盆腔，也可能包含一些中胚层和神经外胚层成分。相比之下，中枢神经系统的神经肠囊肿非常罕见，最常见于椎管内。脊髓内神经肠囊肿通常见于颈胸段，位于硬膜内、髓外，常与上方皮肤和/或椎体的先天性缺陷相关。颅内神经肠囊肿非常少见，典型地位于后颅窝。已经提出了几种假说来解释颅内神经肠囊肿的起源。然而，这些迷人病变的胚胎学基础仍未完全明了。幕上神经肠囊肿极为罕见，术前神经影像学检查常构成诊断挑战。事实上，文献中仅报道了22例幕上神经肠囊肿，包括我们自己诊治的1例以侧方凸面硬膜外囊肿表现为癫痫发作的患者。在本报告中，我们回顾幕上神经肠囊肿的临床、影像学及组织学表现。我们讨论这些有趣病变处理中的鉴别诊断及手术考量。我们还广泛回顾了正常人类胚胎发育过程，并探讨幕上神经肠囊肿胚胎发病机制的推测。
