Laviv Yosef, Jackson Steven, Rappaport Zvi Harry
Beilinson hospital, Rabin Medical Center, Neurosurgery Rabin Medical Center City, Petah Tiqva, Israel, 49100,
Acta Neurochir (Wien). 2015 Feb;157(2):241-5. doi: 10.1007/s00701-014-2309-0. Epub 2014 Dec 19.
Subependymal giant cell tumor (SGCT) is a benign intraventricular tumor, usually located near the foramen of Monro. It is almost always associated with tuberous sclerosis complex (TSC). SGCTs may obstruct cerebrospinal fluid (CSF) pathways. Rarely, they may secrete a protein-rich exudate, causing communicating hydrocephalus. Surgery is indicated for symptomatic lesions or growing asymptomatic lesions. The operative approach to SGCT has shifted from simple shunt placement to a more aggressive approach, leading to early attempts at gross total resection. Recently, the mTOR inhibitor everolimus has been approved for treating SGCT. In this article, we present two cases of recurrent shunt malfunctions in adult TSC patients with protein-secreting SGCTs. We describe the complexity of treating such patients with an emphasis on the role mTOR inhibitors may have in their management. We also review the literature on surgical management of SGCT-related hydrocephalus.
室管膜下巨细胞星形细胞瘤（SGCT）是一种良性脑室内肿瘤，通常位于孟氏孔附近。它几乎总是与结节性硬化症（TSC）相关。SGCT可能阻塞脑脊液（CSF）通路。极少数情况下，它们可能分泌富含蛋白质的渗出液，导致交通性脑积水。对于有症状的病变或不断增大的无症状病变，建议进行手术治疗。SGCT的手术方法已从单纯的分流置管转变为更积极的方法，导致早期尝试进行全切除。最近，mTOR抑制剂依维莫司已被批准用于治疗SGCT。在本文中，我们介绍了两例患有分泌蛋白质的SGCT的成年TSC患者出现分流反复故障的病例。我们描述了治疗此类患者的复杂性，重点强调了mTOR抑制剂在其管理中可能发挥的作用。我们还回顾了关于SGCT相关脑积水手术管理的文献。
