Clinical Outcomes for Pleomorphic Xanthoastrocytoma Patients: an Institutional Experience.

NEURO-ONCOLOGY PRACTICE(2024)

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摘要
Purpose Report our institutional experience with pleomorphic xanthoastrocytoma (PXA) to contribute to limited data on optimal management. Methods Patients with pathologically confirmed PXA treated at our institution between 1990 and 2019 were identified. Demographic information, tumor grade, treatment variables, and clinical outcomes were collected from patient charts. Kaplan-Meier estimates were used to summarize two primary outcome measurements: progression-free survival (PFS) and overall survival (OS). Outcomes were stratified by tumor grade and extent of resection. Cox regression and log-rank testing were performed. Results We identified 17 patients with pathologically confirmed PXA. Two patients were excluded due to incomplete treatment information or < 6m of follow-up; 15 patients were analyzed (median follow-up 4.4y). Six patients had grade 2 PXA and 9 had grade 3 anaplastic PXA. The 2-year and 5-year PFS for the cohort was 57% and 33%, respectively; 2-year and 5-year OS was 93% and 75%, respectively. Patients with grade 2 tumors exhibited superior PFS compared to those with grade 3 tumors (2-year PFS: 100% vs. 28%, 5-year PFS: 60% vs. 14%), hazard ratio, 5.09 (95% CI:1.06–24.50), p = 0.02. Undergoing a GTR also yielded improved outcomes (hazard ratio: 0.38, p = 0.15). All but one (89%) of the grade 3 patients underwent RT. Conclusion The poor survival of the cohort, especially with grade 3 tumors, suggests the need for more aggressive treatment, including maximal resection followed by intensive adjuvant therapy. Better prognostics of tumor recurrence are needed to guide the use of adjuvant therapy.
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关键词
anaplastic,BRAF V600E mutation,pleomorphic xanthoastrocytoma,radiotherapy,surgery
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