Purpose

As per the 2016 World Health Organization (WHO) guidelines on the classification of central nervous system tumors, solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) constitute a single disease entity, known as SFT/HPC. This study provides a clinical analysis of these tumors and describes the treatment outcomes of SFT/HPCs.

Methods

This retrospective study included 76 patients with histopathologically proven SFT/HPC. Reclassification according to the 2016 WHO guideline was done for patients who were diagnosed with SFT or HPC based on the 2007 WHO classification. Recurrence-free survival (RFS) and overall survival (OS) were evaluated for all patients and subgroups.

Results

The median follow-up period was 77.9 months. The median RFS and OS were 126.5 and 136.8 months, respectively. The 1-, 5-, 10-, and 15-year RFS rates were 93%, 72%, 40%, and 40%, respectively. The 1-, 5-, 10- and 15-year OS rates were 97%, 89%, 54%, and 35%, respectively. In multivariable analyses, stereotactic radiosurgery (SRS; p = 0.009, hazard ratio [HR] 6.986), female sex (p = 0.023, HR 1.76), and age over 45 (p = 0.037, HR 2.74) were associated with shorter RFS. Patients who underwent SRS as initial treatment had a shorter OS than that of patients who underwent primary resection (p < 0.001, HR 12.86).

Conclusions

High-grade tumors tended to have worse OS and occur extracranial metastases earlier than low-grade tumors. The median RFS was not different between grade II and III tumors. Male sex, younger age, and GTR were associated with a better RFS. A history of SRS before tumor resection was associated with a shorter RFS and OS.

中文翻译：

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颅内孤立性纤维瘤/血管外皮细胞瘤：肿瘤重新分类和通过 2016 年 WHO 分类评估治疗结果

目的

根据 2016 年世界卫生组织 (WHO) 关于中枢神经系统肿瘤分类的指南，孤立性纤维瘤 (SFT) 和血管外皮细胞瘤 (HPC) 构成一个单一的疾病实体，称为 SFT/HPC。本研究对这些肿瘤进行了临床分析，并描述了 SFT/HPC 的治疗结果。

方法

这项回顾性研究包括 76 名经组织病理学证实的 SFT/HPC 患者。根据 2016 年 WHO 指南对根据 2007 年 WHO 分类诊断为 SFT 或 HPC 的患者进行重新分类。评估了所有患者和亚组的无复发生存期 (RFS) 和总生存期 (OS)。

结果

中位随访时间为 77.9 个月。中位 RFS 和 OS 分别为 126.5 和 136.8 个月。1 年、5 年、10 年和 15 年的 RFS 率分别为 93%、72%、40% 和 40%。1 年、5 年、10 年和 15 年 OS 率分别为 97%、89%、54% 和 35%。在多变量分析中，立体定向放射外科手术（SRS；p  = 0.009，风险比 [HR] 6.986）、女性（p  = 0.023，HR 1.76）和 45 岁以上（p  = 0.037，HR 2.74）与较短的 RFS 相关。接受 SRS 作为初始治疗的患者的 OS 短于接受原发性切除术的患者（p  < 0.001，HR 12.86）。

结论

与低级别肿瘤相比，高级别肿瘤往往具有更差的 OS，并且更早发生颅外转移。II级和III级肿瘤的中位RFS没有差异。男性、年轻和 GTR 与更好的 RFS 相关。肿瘤切除前的 SRS 病史与较短的 RFS 和 OS 相关。