Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients
Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients
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DOI：
10.1007/s00381-019-04449-w
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发表时间：
2020-05-01
影响因子：
1.4
通讯作者：
Genitori, Lorenzo
中科院分区：
文献类型：
--
作者：
Giordano, Flavio;Moscheo, Carla;Genitori, Lorenzo
背景：室管膜下巨细胞星形细胞瘤（SEGA）是结节性硬化症（TSC）的良性肿瘤，可引起脑积水。目前有多种治疗方法，如mTOR抑制剂或手术。手术仍然是一个有效的选择，特别是对于有症状的和较大的肿瘤。方法1994年1月至2015年12月，31例患有SEGA的TSC患者在佛罗伦萨Meyer儿科医院神经外科接受手术治疗。手术指征是肿瘤的大小和位置，生长和囊化/出血，以及脑积水。分析临床资料、术前、术后MRI、复发率、进一步手术及相关并发症。结果31例受SEGA影响的TSC患者共行44次手术，分别实现36例（GTR）和8例（STR）全切除。11例患者复发；其中9人接受了进一步的手术治疗，2人接受了mTOR通路抑制剂治疗。手术发病率和死亡率分别为22.7%和2.3%。平均随访4.9年，90%的患者无肿瘤，93.3%的患者神经系统状态良好；12例（40%）因脑积水行脑室-腹腔分流术（VPS）。结论：本系列研究证实，手术入路联合mTOR抑制剂仍然是治疗SEGAs的有效选择。
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.