Ida Cristiane M, Rodriguez Fausto J, Burger Peter C, Caron Alissa A, Jenkins Sarah M, Spears Grant M, Aranguren Dawn L, Lachance Daniel H, Giannini Caterina
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Department of Pathology, Johns Hopkins University, Baltimore, MD, USA.
Brain Pathol. 2015 Sep;25(5):575-86. doi: 10.1111/bpa.12217. Epub 2014 Dec 5.
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 ≥ 5/10 HPF 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 ≥ 5/10 HPF 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 ≥ 5/10 HPF. 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.
回顾性评估了74例多形性黄色星形细胞瘤（PXA）患者组织学间变和BRAF V600E突变的预后意义。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（PXA-AF），定义为有丝分裂指数≥5/10高倍视野和/或存在坏死。通过免疫组化和/或分子分析在60例中的39例检测到BRAF V600E突变，所有病例IDH1（R132H）均为阴性。有丝分裂指数≥5/10高倍视野和坏死与总生存期（OS）降低相关（分别为P = 0.0005和P = 0.0002）。除2例之外，所有病例中坏死均与有丝分裂指数≥5/10高倍视野相关。与无BRAF V600E突变的患者相比，BRAF V600E突变肿瘤患者的OS显著更长（P = 0.02）。无论年龄如何，PXA-AF患者的OS均显著短于无PXA-AF的患者（P = 0.0003）。仅成人PXA-AF患者的无复发生存期显著更短（P = 0.047）。与未复发或未死亡的患者相比，诊断后≤3年复发或死亡的患者在首次诊断时更有可能患有PXA-AF（P = 0.008）或接受了非根治性切除手术（P = 0.004）。本研究提供了进一步的证据，表明PXA-AF的行为比PXA更具侵袭性，可能符合世界卫生组织III级“间变性”的命名。
