Torres O A, Roach E S, Delgado M R, Sparagana S P, Sheffield E, Swift D, Bruce D
Division of Pediatric Neurology, University of Texas Southwestern Medical School, Children's Medical Center of Dallas, 75235, USA.
J Child Neurol. 1998 Apr;13(4):173-7. doi: 10.1177/088307389801300405.
We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence.
我们报告了19例患有结节性硬化症合并室管膜下巨细胞星形细胞瘤的患者。肿瘤诊断时的平均年龄为9.4岁（范围为1.5至21岁）。头颅计算机断层扫描（CT）或头颅磁共振成像（MRI）确定了病变，所有病例均进行了切除。7例患者有脑积水，12例患者肿瘤大小有间隔性增加或有大肿瘤但无脑积水。手术标准包括：（1）存在脑积水；（2）肿瘤大小间隔性增加；（3）可归因于肿瘤的新的局灶性神经功能缺损；和/或（4）颅内压升高的症状。8例患者是通过包括每年进行头颅计算机断层扫描的监测项目发现的。这8例患者在症状出现前均切除了肿瘤，术后均无持续的神经功能缺损，且迄今为止均未发生复发性室管膜下巨细胞星形细胞瘤。相比之下，在非监测组的11例患者中，7例在肿瘤诊断时有症状，1例术后病程复杂，2例发生复发性巨细胞星形细胞瘤，1例有广泛病变无法完全切除。定期头颅影像学检查可能有助于在结节性硬化症患者出现症状前识别室管膜下巨细胞星形细胞瘤。早期诊断和治疗可降低手术并发症发生率和肿瘤复发风险。
