■高级别多形性黄色星形细胞瘤(HGPXAs)非常罕见,其治疗和预后结果尚不清楚。为了更好地了解疾病,我们旨在评估无进展生存期(PFS)和总生存期(OS)的风险因素,并根据我们研究所的病例和文献中的病例提出治疗方案。
■作者回顾了2014年8月至2021年9月在北京天坛医院神经外科接受手术治疗的26例HGPXAs患者的临床资料。我们还在1997年1月至2022年10月之间使用关键词“间变性”和“多形性黄色星形细胞瘤”搜索了PubMed数据库。在合并病例中评估了PFS和OS的危险因素。
■作者的队列包括11名男性和15名女性,平均年龄为36.7±20.3岁(范围:5.5-71岁)。17例(65.4%)和9例(34.6%)患者实现了大体全切除(GTR)和非GTR,分别。对22和20例患者进行了放疗和化疗,分别。平均随访20.5±21.2个月(范围:0.5-78.1个月),肿瘤复发7例,死亡6例,平均OS时间为19.4±10.8个月(范围:8-36个月)。在1997年1月至2022年10月的文献中,在29名男性和27名女性中发现了56例HGPXAs,平均年龄为29.6±19.6岁(范围;4-74岁)。其中,24例(44.4%)患者取得GTR。对31例(62%)患者和23例(46%)患者进行了放疗和化疗,分别。中位随访31.4±35.3个月(范围:0.75-144个月)后,死亡率和复发率分别为32.5%(13/40)和70%(28/40),分别。多变量Cox回归模型显示非GTR(HR0.380,95%CI0.174-0.831,p=0.015),年龄≥30岁(HR2.620,95%CI1.183-5.804,p=0.018),无RT(HR0.334,95%CI0.150-0.744,p=0.007)和无CT(HR0.422,95%CI0.184-0.967,p=0.042)是PFS的阴性预后因素。非GTR(HR0.126,95%CI0.037-0.422,p=0.001),继发性HGPXAs(HR7.567,95%CI2.221-25.781,p=0.001),年龄≥30岁(HR3.568,95%CI1.190-10.694,p=0.023)和无RT(HR0.223,95%CI0.073-0.681,p=0.008)是OS的危险因素.
■高级多形性黄色星形细胞瘤是非常罕见的脑肿瘤。儿童和年轻人比老年患者有更好的临床结果。次要HGPXAs的OS比主要HGPXAs差。建议对该实体进行完整的手术切除加RT和CT。本研究中HGPXAs的BRAF突变频率为47.5%(19/40),然而,我们没有发现BRAF突变与临床结局之间的联系.未来有必要进行更大的队列研究来验证我们的发现。
UNASSIGNED: High grade pleomorphic xanthoastrocytomas (HGPXAs) are very rare and their management and prognostic outcomes remain unclear. To better understand the disease, we aimed to evaluate the risk factors for progression-free survival (PFS) and overall survival (OS), and propose a treatment protocol based on cases from our institute and cases from the literature.
UNASSIGNED: The authors reviewed the clinical data of 26 patients with HGPXAs who underwent surgical treatment in Department of Neurosurgery of Beijing Tiantan Hospital between August 2014 and September 2021. We also searched the PubMed database using the keywords \"anaplastic\" combined with \"pleomorphic xanthoastrocytoma(s)\" between January 1997 and October 2022. Risk factors for PFS and OS were evaluated in the pooled cases.
UNASSIGNED: The authors\' cohort included 11 males and 15 females with a mean age of 36.7 ± 20.3 years (range: 5.5-71 years). Gross-total resection (GTR) and non-GTR were achieved in 17 (65.4%) and 9 (34.6%) patients, respectively. Radiotherapy and chemotherapy were administered to 22 and 20 patients, respectively. After a mean follow-up of 20.5 ± 21.2 months (range: 0.5-78.1 months), 7 patients suffered tumor recurrence and 6 patients died with a mean OS time of 19.4 ± 10.8 months (range: 8-36 months). In the literature between January 1997 and October 2022, 56 cases of HGPXAs were identified in 29 males and 27 females with a mean age of 29.6 ± 19.6 years (range; 4-74 years). Among them, 24 (44.4%) patients achieved GTR. Radiotherapy and chemotherapy was administered to 31 (62%) patients and 23 (46%) patients, respectively. After a median follow-up of 31.4 ± 35.3 months (range: 0.75-144 months), the mortality and recurrence rates were 32.5% (13/40) and 70% (28/40), respectively. Multivariate Cox regression model demonstrated that non-GTR (HR 0.380, 95% CI 0.174-0.831, p=0.015), age≥30 (HR 2.620, 95% CI 1.183-5.804, p=0.018), no RT (HR 0.334,95% CI 0.150-0.744, p=0.007) and no CT (HR 0.422, 95% CI 0.184-0.967, p=0.042) were negative prognostic factors for PFS. Non-GTR (HR 0.126, 95% CI 0.037-0.422, p=0.001), secondary HGPXAs (HR 7.567, 95% CI 2.221-25.781, p=0.001), age≥30 (HR 3.568, 95% CI 1.190-10.694, p=0.023) and no RT (HR 0.223,95% CI 0.073-0.681, p=0.008) were risk factors for OS.
UNASSIGNED: High grade pleomorphic xanthoastrocytomas are very rare brain tumors. Children and younger adults have better clinical outcome than elderly patients. Secondary HGPXAs had worse OS than primary HGPXAs. Complete surgical excision plus RT and CT is recommended for this entity. The frequency of BRAF mutations in HGPXAs is 47.5% (19/40) in this study, however, we do not find the connections between BRAF mutations and clinical outcomes. Future studies with larger cohorts are necessary to verify our findings.