Pleomorphic Xanthoastrocytoma: Natural History and Long-Term Follow-Up.
Pleomorphic Xanthoastrocytoma: Natural History and Long-Term Follow-Up.
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DOI：
10.1111/bpa.12217
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发表时间：
2015-09
期刊：
影响因子：
--
通讯作者：
Giannini C
中科院分区：
文献类型：
--
作者：
Ida CM;Rodriguez FJ;Burger PC;Caron AA;Jenkins SM;Spears GM;Aranguren DL;Lachance DH;Giannini C
对74例多形性黄色星形细胞瘤（PXA）患者的组织学间变性和BRAF V600 E突变的预后意义进行了回顾性评价。诊断时的中位年龄为21.5岁（31例儿童，43例成人），中位随访时间为7.6年。33例存在间变性（PXA AF），定义为核分裂指数≥ 5/10 HPF和/或存在坏死。免疫组化和/或分子生物学分析检测到39例BRAF V600 E突变，均为IDH 1（R132 H）阴性。有丝分裂指数≥ 5/10 HPF和坏死与总生存率降低相关（OS;分别为P = 0.0005和P = 0.0002）。除2例外，所有病例的坏死均与核分裂指数≥ 5/10 HPF相关。BRAF V600 E突变肿瘤患者的OS显著长于无BRAF V600 E突变的患者（P = 0.02）。PXA-AF患者，无论年龄大小，与无PXA-AF患者相比，OS显著缩短（P = 0.0003）。仅成人PXA-AF患者的无复发生存期显著较短（P = 0.047）。复发或诊断后≤3年内死亡的患者与未接受PXA-AF的患者相比，首次诊断时更可能患有PXA-AF（P = 0. 008）或接受非大体全切除术（P = 0. 004）。这项研究提供了进一步的证据表明，PXA-AF的行为更具侵略性比PXA，并可能符合WHO III级“间变性”指定。
Prognostic significance of histological anaplasia and BRAF V600E mutation were retrospectively evaluated in 74 patients with pleomorphic xanthoastrocytoma (PXA). Median age at diagnosis was 21.5 years (31 pediatric, 43 adult) and median follow-up 7.6 years. Anaplasia (PXA-AF), defined as mitotic index ≥ 5/10HPF and/or presence of necrosis, was present in 33 cases. BRAF V600E mutation was detected in 39 (of 60) cases by immunohistochemical and/or molecular analysis, all negative for IDH1 (R132H). Mitotic index ≥ 5/ 10HPF and necrosis were associated with decreased overall survival (OS; P = 0.0005 and P = 0.0002, respectively). In all cases except two, necrosis was associated with mitotic index ≥ 5/10HPF. Patients with BRAF V600E mutant tumors had significantly longer OS compared with those without BRAF V600E mutation (P = 0.02). PXA-AF patients, regardless of age, had significantly shorter OS compared with those without (P = 0.0003). Recurrence-free survival was significantly shorter for adult PXA-AF patients (P = 0.047) only. Patients who either recurred or died ≤3 years from diagnosis were more likely to have had either PXA-AF at first diagnosis (P = 0.008) or undergone a non-gross total resection procedure (P = 0.004) as compared with patients who did not. This study provides further evidence that PXA-AF behaves more aggressively than PXA and may qualify for WHO grade III “anaplastic” designation.
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影响因子：
15.9
作者：
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通讯作者：
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作者：
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作者：
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通讯作者：
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影响因子：
1.4
作者：
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通讯作者：
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