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小儿促纤维增生性星形细胞瘤:美国国家数据库对当前治疗方法的调查。

Pediatric Pleomorphic Xanthoastrocytoma: A National Database Inquiry on Current Treatment Approaches in the United States.

机构信息

Department of Radiation Medicine, Oregon Health & Science University, Portland, Oregon, USA.

Biostatistics Shared Resource, Oregon Health & Science University, Portland, Oregon, USA.

出版信息

Cancer Rep (Hoboken). 2021 Dec;4(6):e1415. doi: 10.1002/cnr2.1415. Epub 2021 May 8.

Abstract

BACKGROUND

Pleomorphic xanthoastrocytomas (PXAs) account for <1% of primary brain tumors, occurring predominantly in children and young adults. Surgical resection serves as the primary treatment for PXAs, while radiotherapy (RT) and chemotherapy protocols remain poorly defined.

AIM

This study aims to determine current care patterns utilized for pediatric patients (≤ 18 years) diagnosed with PXAs and their effect on overall survival.

METHODS

The United States National Cancer Database (NCDB) was queried between 2004 and 2015 for pediatric patients (≤18 years) diagnosed with PXAs.

RESULTS

From the 224 qualifying patients, most patients proceeded with surgery only (78.1%), while 11.6% of patients received both adjuvant RT and chemotherapy. In the 2010-2015 cohort, patients with subtotal resection were associated with poorer prognosis than those with gross-total resection (hazard ratio = 17.44, 95% confidence interval = 2.10-144.90, p < .001). RT and chemotherapy recipients were similarly associated with poorer survival than those treated with surgery only, with p-values of <.001 and respective hazard ratios of 3.82 (95% confidence interval = 1.85-7.90) and 6.68 (95% confidence interval = 3.21-13.89). The key factors impacting the probability of RT delivery involved WHO grade (p < .001) and chemotherapy administration (p < .001). However, WHO grade alone did not significantly impact survival (p-value = .088).

CONCLUSION

Maximally safe resection is the current treatment goal for patients with PXAs. RT and chemotherapy are poorly utilized but had a greater role in managing more aggressive cases of PXAs. Additional research focusing on the impact of adjuvant therapies on tumor progression is needed to better guide treatment decisions.

摘要

背景

多形性黄色星形细胞瘤(PXAs)占原发性脑肿瘤的<1%,主要发生在儿童和年轻成人中。手术切除是 PXA 的主要治疗方法,而放疗(RT)和化疗方案仍未得到明确界定。

目的

本研究旨在确定目前用于诊断为 PXA 的儿科患者(≤18 岁)的治疗模式及其对总生存率的影响。

方法

2004 年至 2015 年期间,美国国家癌症数据库(NCDB)对诊断为 PXA 的儿科患者(≤18 岁)进行了查询。

结果

在 224 名符合条件的患者中,大多数患者仅接受手术治疗(78.1%),而 11.6%的患者接受了辅助 RT 和化疗。在 2010-2015 年队列中,次全切除患者的预后比大体全切除患者差(风险比=17.44,95%置信区间=2.10-144.90,p<.001)。接受 RT 和化疗的患者的生存率也明显低于仅接受手术治疗的患者,p 值均<.001,相应的风险比分别为 3.82(95%置信区间=1.85-7.90)和 6.68(95%置信区间=3.21-13.89)。影响 RT 治疗可能性的关键因素包括世界卫生组织(WHO)分级(p<.001)和化疗管理(p<.001)。然而,仅 WHO 分级并不显著影响生存(p 值=.088)。

结论

最大限度地安全切除是 PXA 患者的当前治疗目标。RT 和化疗的应用不足,但在治疗更具侵袭性的 PXA 病例方面发挥了更大的作用。需要进一步研究辅助治疗对肿瘤进展的影响,以更好地指导治疗决策。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c62/8714547/4f78175a268c/CNR2-4-e1415-g001.jpg

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