A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex Clinical article
A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex Clinical article
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DOI：
10.3171/2013.9.peds13193
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发表时间：
2014-01-01
影响因子：
1.9
通讯作者：
Weiner, Howard L.
中科院分区：
文献类型：
--
作者：
Harter, David H.;Bassani, Luigi;Weiner, Howard L.
对象。室管膜下巨细胞星形细胞瘤（SEGAs）是一种良性肿瘤，最常见于结节性硬化症（TSC）。这些肿瘤绝大多数发生在靠近Monro孔的外侧室管膜表面，因此可能侵犯一个或两个孔，导致梗阻性脑积水，需要手术减压。本文介绍了手术适应证、术中注意事项以及作者管理模式的演变。1997年1月至2011年3月期间在纽约大学朗格尼医学中心接受开颅手术进行SEGA切除术的TSC患者被确定。我们回顾了术前影像学、临床特征、管理决策、手术程序和结果。18例TSC患者接受了22例原发性SEGAs小切除术。手术指征为21例中16例有意义的影像学肿瘤进展。手术时的平均年龄为10.3岁。平均随访时间52个月（12-124个月）。手术入路16例为脑内经胼胝体，5例为脑皮质经脑室，1例为神经内窥镜。右侧肿瘤9例，左侧肿瘤9例，双侧肿瘤3例。在我们的研究中，22例患者中有16例进行了总全切除术，4例进行了根治性次全切除术，2例进行了次全切除术。2例患者术前行脑室腹腔分流术，7例患者因中重度脑室肿大术后需行分流术。2例患者肿瘤进展需要再次手术；这两例患者最初都接受了str。作者介绍了他们对TSC合并SEGAs患者的管理策略。选择发生率可接受的患者行显微外科手术切除SEGAs。90.9%的患者（22例原发肿瘤切除术中的20例）实现了总切除或根治性STR，该组无复发。大约有一半的患者需要脑脊液转移手术。没有一例与手术相关的永久性神经系统疾病。
Object. Subependymal giant cell astrocytomas (SEGAs) are benign tumors, most commonly associated with tuberous sclerosis complex (TSC). The vast majority of these tumors arise from the lateral ependymal surface adjacent to the foramen of Monro, therefore potentially encroaching on one or both foramina, and resulting in obstructive hydrocephalus that necessitates surgical decompression. The indications for surgery, intraoperative considerations, and evolution of the authors' management paradigm are presented.Methods. Patients with TSC who underwent craniotomy for SEGA resection at New York University Langone Medical Center between January 1997 and March 2011 were identified. Preoperative imaging, clinical characteristics, management decisions, operative procedures, and outcomes were reviewed.Results. Eighteen patients with TSC underwent 22 primary minor resections for SEGAs. The indication for surgery was meaningful radiographic tumor progression in 16 of 21 cases. The average age at the time of operation was 10.3 years. Average follow-up duration was 52 months (range 12-124 months). The operative approach was intrahemispheric-transcallosal in 16 cases, transcortical-transventricular in 5, and neuroendoscopic in 1. Nine tumors were on the right, 9 on the left, and 3 were bilateral. Gross-total resection was documented in 16 of 22 cases in our series, with radical subtotal resection achieved in 4 cases, and subtotal resection (STR) in 2 cases. Two patients had undergone ventriculoperitoneal shunt placement preoperatively and 7 patients required shunt placement after surgery for moderate to severe ventriculomegaly. Two patients experienced tumor progression requiring reoperation; both of these patients had initially undergone STR.Conclusions. The authors present their management strategy for TSC patients with SEGAs. Select patients underwent microsurgical resection of SEGAs with acceptable morbidity. Gross-total resection or radical STR was achieved in 90.9% of our series (20 of 22 primary tumor resections), with no recurrences in this group. Approximately half of our patient series required CSF diversionary procedures. There were no instances of permanent neurological morbidity associated with surgery.