中枢神经系统孤立性纤维瘤/血管外皮细胞瘤的临床结局：一项符合 STROBE 规范的单中心分析。
Clinical outcomes in central nervous system solitary-fibrous tumor/hemangiopericytoma: a STROBE-compliant single-center analysis.
机构信息
Department of Neurosurgery, Pituitary Adenoma Multidisciplinary Center, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu, 610041, China.
出版信息
World J Surg Oncol. 2022 May 10;20(1):149. doi: 10.1186/s12957-022-02619-w.
BACKGROUND
Solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) are rare mesenchymal tumors in the central nervous system with a high tendency to relapse, having a significant impact on quality of life (QoL). Due to the rarity of intracranial SFT/HPC, the prognostic factors and optimal treatment remain to be elucidated. Meanwhile, quality of life in patients with intracranial SFT/HPC is seldomly concerned. Thus, we aim to survey about the quality of life and underline some aspects demanding concern in intracranial SFT/HPC treatment through summarizing our case series in recent ten years.
METHODS
Patients with intracranial SFT/HPC who underwent surgical resection from January 2009 to June 2019 were included in the study. Clinical features, such as age, gender, and resection extent, were collected. The EuroQol Five Dimensions Questionnaire (EQ-5D) was used to assess the patients' quality of life (QoL). Prognosis factors related to progression-free survival (PFS) and overall survival (OS) were also evaluated.
RESULTS
Thirty-six patients with a mean follow-up period of 61.6 months (range 13-123 months) were included in this study. Sixteen (44.4%) patients achieved gross total resection (GTR). Fourteen patients (38.9%) with tumor progression experienced adjuvant radiotherapy (11.1%) or Gamma Knife surgery (GKS, 27.8%). According to the 2016 WHO classification, there were 6 (16.7%) grade I SFT/HPC, 11 (30.5%) grade II SFT/HPC, and 19 (52.8%) grade III SFT/HPC. The PFS and OS were 29 months (range 4-96 months) and 38 months (range 4-125 months). The median EQ5D-3 L tariff with or without progression was 0.617 (95% CI 0.470-0.756) and 0.939 (95% CI 0.772-0.977) respectively. Gross total resection (GTR, p = 0.024) and grade I SFT/HPC (p = 0.017) were significantly associated with longer PFS. In multivariate analysis, GTR (HR 0.378, 95% CI 0.154-0.927) and adjuvant therapy (HR 0.336, 95% CI 0.118-0.956) result in significantly longer PFS in patients with SFT/HPC.
CONCLUSIONS
Patients underwent GTR and adjuvant therapy had longer PFS. Similarly, patients with lower WHO grade had relatively longer PFS. Therefore, GTR is advocated for the treatment of SFT/HPC. And adjuvant therapy such as GKS could be an alternative treatment for patients who underwent STR or with tumor progression. Further, the QoL decreased in patients with tumor progression and metastasis, and more attention is demanded to the QoL of intracranial SFT/HPC patients.
背景
孤立性纤维瘤（SFT）和血管外皮细胞瘤（HPC）是中枢神经系统中罕见的间叶性肿瘤，具有较高的复发倾向，对生活质量（QoL）有重大影响。由于颅内 SFT/HPC 较为罕见，其预后因素和最佳治疗方法仍有待阐明。同时，颅内 SFT/HPC 患者的生活质量很少受到关注。因此，我们旨在通过总结近十年来的病例系列，调查颅内 SFT/HPC 患者的生活质量，并强调颅内 SFT/HPC 治疗中需要关注的一些方面。
方法
纳入 2009 年 1 月至 2019 年 6 月期间接受手术切除的颅内 SFT/HPC 患者。收集年龄、性别和切除程度等临床特征。采用欧洲五维健康量表（EQ-5D）评估患者的生活质量（QoL）。还评估了与无进展生存期（PFS）和总生存期（OS）相关的预后因素。
结果
本研究纳入了 36 例平均随访时间为 61.6 个月（范围 13-123 个月）的患者。16 例（44.4%）患者达到了大体全切除（GTR）。14 例（38.9%）肿瘤进展患者接受了辅助放疗（11.1%）或伽玛刀手术（GKS，27.8%）。根据 2016 年 WHO 分级，有 6 例（16.7%）为 I 级 SFT/HPC，11 例（30.5%）为 II 级 SFT/HPC，19 例（52.8%）为 III 级 SFT/HPC。PFS 和 OS 分别为 29 个月（范围 4-96 个月）和 38 个月（范围 4-125 个月）。无进展和有进展时的 EQ5D-3L 税则中位数分别为 0.617（95%CI 0.470-0.756）和 0.939（95%CI 0.772-0.977）。大体全切除（GTR，p=0.024）和 I 级 SFT/HPC（p=0.017）与较长的 PFS 显著相关。多变量分析显示，GTR（HR 0.378，95%CI 0.154-0.927）和辅助治疗（HR 0.336，95%CI 0.118-0.956）可显著延长 SFT/HPC 患者的 PFS。
结论
GTR 和辅助治疗可延长 SFT/HPC 患者的 PFS。同样，WHO 分级较低的患者 PFS 也相对较长。因此，GTR 是治疗 SFT/HPC 的首选方法。对于接受 STR 或肿瘤进展的患者，GKS 等辅助治疗可能是一种替代治疗方法。此外，肿瘤进展和转移的患者生活质量下降，需要更加关注颅内 SFT/HPC 患者的生活质量。
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