BACKGROUND Subependymal giant cell astrocytomas (SEGA) are benign tumors characteristic of tuberous sclerosis complex (TSC) that may cause hydrocephalus. Various treatments are nowadays available as mTOR inhibitors or surgery. Surgery is still a valid option especially for symptomatic and larger tumors. METHODS From January 1994 to December 2015, 31 TSC patients harboring SEGA underwent surgery at the Department of Neurosurgery of the Meyer Pediatric Hospital, Florence. Indications for surgery were tumor size and location, growth and cystization/hemorrhage, and hydrocephalus. Clinical data, preoperative and postoperative MRI, recurrence rate, further surgical procedures, and related complications were analyzed. RESULTS A total of 44 surgeries were performed in 31 TSC patients affected by SEGA, achieving gross total removal (GTR) and subtotal removal (STR), respectively, in 36 and 8 patients. Recurrences occurred in 11 patients; 9 of them underwent further surgical procedures and 2 were treated with mTOR pathway inhibitors. Surgical morbidity and mortality were, respectively, 22.7% and 2.3%. After a mean follow-up of 4.9 years, 90% of patients were tumor-free with good neurological status in 93.3%; twelve (40%) had a ventriculo-peritoneal shunt (VPS) for hydrocephalus. CONCLUSIONS The present series confirms that the surgical approach, combined with mTOR inhibitors, is still a valid option for the treatment of SEGAs.

中文翻译：

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神经外科治疗结节性硬化症中的室管膜下巨细胞星形细胞瘤：31例患者的44例手术。

背景技术室管膜下巨细胞星形细胞瘤（SEGA）是结节性硬化复合物（TSC）的良性肿瘤，可能导致脑积水。如今，有多种治疗手段可作为mTOR抑制剂或手术治疗。手术仍然是有效的选择，尤其是对于有症状的和较大的肿瘤。方法从1994年1月至2015年12月，31名患有SEGA的TSC患者在佛罗伦萨Meyer儿科医院神经外科进行了手术。手术的指征是肿瘤的大小和位置，生长和囊肿/出血以及脑积水。临床数据，术前和术后MRI，复发率，进一步的手术程序和相关的并发症进行了分析。结果共对31例受SEGA影响的TSC患者进行了44次手术，分别在36和8位患者中实现了总总清除量（GTR）和小计清除量（STR）。11例患者复发。其中9例接受了进一步的外科手术，其中2例使用mTOR途径抑制剂治疗。手术发病率和死亡率分别为22.7％和2.3％。平均随访4.9年后，90％的患者无肿瘤，神经系统状态良好，占93.3％；12例（40％）患有脑积水的心室-腹膜分流（VPS）。结论本系列研究证实，手术方法结合mTOR抑制剂仍然是治疗SEGA的有效选择。分别为22.7％和2.3％。平均随访4.9年后，90％的患者无肿瘤，神经系统状态良好，占93.3％；12例（40％）患有脑积水的心室-腹膜分流（VPS）。结论本系列研究证实，手术方法结合mTOR抑制剂仍然是治疗SEGA的有效选择。分别为22.7％和2.3％。平均随访4.9年后，90％的患者无肿瘤，神经系统状态良好，占93.3％；12例（40％）患有脑积水的心室-腹膜分流（VPS）。结论本系列研究证实，手术方法结合mTOR抑制剂仍然是治疗SEGA的有效选择。