Zhang Dong-Yong, Su Lan, Wang Yi-Wei
Department of Neurosurgery, the First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China.
Department of Genetron Health, Genetron Health, Beijing 110024, China.
World J Clin Cases. 2022 Jan 14;10(2):631-642. doi: 10.12998/wjcc.v10.i2.631.
Solitary fibrous tumor (SFT) of the central nervous system is rare. It is predominantly benign and rarely malignant. There is no established standardized treatment regimen for malignant intracranial SFTs.
We present a rare case of SFT in a 9-year-old girl with a space-occupying effect in the frontal-parietal lobes. She underwent craniotomy, and the mass was resected. Immunohistochemistry examination of the specimen showed that Ki-67 proliferation index staining was highly positive in 80% of tumor cells. Whole exome sequencing of the surgical tissue showed 38 somatic gene mutations and 1 gene amplification such as fibroblast growth factor receptor 4 or TP53. At 1.5 mo after surgery, head magnetic resonance imaging revealed that the tumor had recurred. The patient received 60 Gy and 30 fractions of intensity modulated radiotherapy. The patient then received anlotinib 8 mg po qd for 1-14 d of a 21 d cycle. Following this regimen, the patient achieved stable disease for > 17 mo. Magnetic resonance imaging at 1.5 year after surgery showed that the tumor had not progressed.
This is the first reported case of SFT of the central nervous system treated with surgery, radiotherapy and anlotinib. This regimen may be an effective treatment option for malignant intracranial SFT patients.
中枢神经系统孤立性纤维瘤（SFT）较为罕见。它主要为良性，恶性者极少。目前尚无针对恶性颅内SFT的既定标准化治疗方案。
我们报告了1例9岁女孩的罕见SFT病例，肿瘤位于额顶叶，具有占位效应。她接受了开颅手术，肿瘤被切除。标本的免疫组织化学检查显示，80%的肿瘤细胞中Ki-67增殖指数染色呈强阳性。手术组织的全外显子测序显示存在38个体细胞基因突变和1个基因扩增，如成纤维细胞生长因子受体4或TP53。术后1.5个月，头部磁共振成像显示肿瘤复发。患者接受了60 Gy、30次分割的调强放疗。然后患者接受安罗替尼8 mg口服，每日1次，每21天为1周期，连用1 - 14天。按照此方案，患者病情稳定超过17个月。术后1.5年的磁共振成像显示肿瘤未进展。
这是首例报告的采用手术、放疗及安罗替尼治疗的中枢神经系统SFT病例。该方案可能是恶性颅内SFT患者的一种有效治疗选择。
