目的:作者旨在探讨根据2021年WHO中枢神经系统(CNS)肿瘤分类重新分类的孤立性纤维性肿瘤(SFTs)和血管外皮细胞瘤(HPCs)的复发和生存相关的临床结果和危险因素。
方法:作者回顾性收集并分析了2007年1月至2021年12月记录的SFT和HPCs的临床和病理资料。两名神经病理学家根据2021年WHO分类重新评估了病理切片和重新分级的标本。通过单变量和多变量Cox回归分析对与无进展生存期(PFS)和总生存期(OS)相关的预后因素进行统计学评估。
结果:共有146名患者(74名男性和72名女性,平均±SD[范围]年龄46.1±14.3[3-78]岁)进行审查,根据2021年WHO分类,86、35和25例患者被重新分类为1、2和3级SFT,分别。WHO1级SFT患者的中位PFS和OS在初次诊断后分别为105个月和199个月;对于WHO2级SFT患者,77个月和145个月;对于WHO3级SFT患者,44个月和112个月,分别。在整个队列中,61例患者出现局部复发,31例死亡,其中27人(87.1%)死于SFT及相关并发症。10例患者发生颅外转移。在多元Cox回归分析中,次全切除术(STR)(HR4.648,95%CI2.601-8.304,p<0.001),位于矢状旁或旁区域的肿瘤(HR2.105,95%CI1.099-4.033,p=0.025),椎骨中的肿瘤(HR3.352,95%CI1.228-9.148,p=0.018),WHO2级SFT(HR2.579,95%CI1.343-4.953,p=0.004),WHO3级SFT(HR5.814,95%CI2.887-11.712,p<0.001)与PFS缩短显著相关,而STR(HR3.217,95%CI1.435-7.210,p=0.005)和WHO3级SFT(HR3.433,95%CI1.324-8.901,p=0.011)与OS缩短显著相关。在单变量分析中,STR后接受辅助放疗(RT)的患者比未接受RT的患者具有更长的PFS.
结论:2021年WHO中枢神经系统肿瘤分类能更好地预测不同病理分级的恶性肿瘤,特别是WHO3级SFT的预后较差。大体全切除(GTR)可以显着延长PFS和OS,应作为最重要的治疗方法。辅助RT对接受STR的患者有帮助,但对接受GTR的患者没有帮助。
OBJECTIVE: The authors aimed to explore the clinical outcomes and risk factors related to recurrence of and survival from solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) that were reclassified according to the 2021 WHO classification of central nervous system (CNS) tumors.
METHODS: The authors retrospectively collected and analyzed the clinical and pathological data of SFTs and HPCs recorded from January 2007 to December 2021. Two neuropathologists reassessed pathological slides and regraded specimens on the basis of the 2021 WHO classification. The prognostic factors related to progression-free survival (PFS) and overall survival (OS) were statistically assessed with univariate and multivariate Cox regression analyses.
RESULTS: A total of 146 patients (74 men and 72 women, mean ± SD [range] age 46.1 ± 14.3 [3-78] years) were reviewed, and 86, 35, and 25 patients were reclassified as having grade 1, 2, and 3 SFTs on the basis of the 2021 WHO classification, respectively. The median PFS and OS of the patients with WHO grade 1 SFT were 105 months and 199 months after initial diagnosis; for patients with WHO grade 2 SFT, 77 months and 145 months; and for patients with WHO grade 3 SFT, 44 months and 112 months, respectively. Of the entire cohort, 61 patients experienced local recurrence and 31 died, of whom 27 (87.1%) died of SFT and relevant complications. Ten patients had extracranial metastasis. In multivariate Cox regression analysis, subtotal resection (STR) (HR 4.648, 95% CI 2.601-8.304, p < 0.001), tumor located in the parasagittal or parafalx region (HR 2.105, 95% CI 1.099-4.033, p = 0.025), tumor in the vertebrae (HR 3.352, 95% CI 1.228-9.148, p = 0.018), WHO grade 2 SFT (HR 2.579, 95% CI 1.343-4.953, p = 0.004), and WHO grade 3 SFT (HR 5.814, 95% CI 2.887-11.712, p < 0.001) were significantly associated with shortened PFS, whereas STR (HR 3.217, 95% CI 1.435-7.210, p = 0.005) and WHO grade 3 SFT (HR 3.433, 95% CI 1.324-8.901, p = 0.011) were significantly associated with shortened OS. In univariate analyses, patients who received adjuvant radiotherapy (RT) after STR had longer PFS than patients who did not receive RT.
CONCLUSIONS: The 2021 WHO classification of CNS tumors better predicted malignancy with different pathological grades, and in particular WHO grade 3 SFT had worse prognosis. Gross-total resection (GTR) can significantly prolong PFS and OS and should serve as the most important treatment method. Adjuvant RT was helpful for patients who underwent STR but not for patients who underwent GTR.