背景:2021年发布的世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类的最新第五版(WHOCNS5分类)定义了星形细胞瘤,IDH-突变体,4年级。然而,对这种亚型的理解仍然有限。我们进行了这项研究来描述星形细胞瘤的特征,IDH-突变体,4级,并探讨了组织学和分子亚型之间的异同。
方法:2011年1月至2022年1月接受手术的患者,分类为星形细胞瘤,IDH-突变体,该研究包括4级。临床,放射学,组织病理学,分子病理学,收集生存数据进行分析.
结果:总共33例星形细胞瘤患者,IDH-突变体,4年级被选中,包括20例组织学和13例分子WHO4级星形细胞瘤。肿瘤增强,肿瘤内坏死样表现,肿瘤周围水肿较大,在组织学WHO4级星形细胞瘤中经常观察到更明确的肿瘤边缘。此外,分子WHO4级星形细胞瘤显示出相对较长的总体生存期的趋势,而没有达到统计学意义(47vs.25个月,p=0.22)。通常观察到TP53、CDK6和PIK3CA改变,而PIK3R1(p=0.033),Notch1(p=0.027),和Mycn(p=0.027)改变可能会影响WHO4级星形细胞瘤的总体生存率。
结论:我们的研究仔细检查了IDH突变体,4级星形细胞瘤。因此,应考虑进一步分类,因为组织学和分子WHO4级星形细胞瘤的预后不同.值得注意的是,针对PIK3R1,Notch1和Mycn的治疗可能是有益的.
The latest fifth edition of the World Health Organization (WHO) classification of the central nervous system (CNS) tumors (WHO CNS 5 classification) released in 2021 defined astrocytoma, IDH-mutant, Grade 4. However, the understanding of this subtype is still limited. We conducted this study to describe the features of astrocytoma, IDH-mutant, Grade 4 and explored the similarities and differences between histological and molecular subtypes.
Patients who underwent surgery from January 2011 to January 2022, classified as astrocytoma, IDH-mutant, Grade 4 were included in this study. Clinical, radiological, histopathological, molecular pathological, and survival data were collected for analysis.
Altogether 33 patients with astrocytoma, IDH-mutant, Grade 4 were selected, including 20 with histological and 13 with molecular WHO Grade 4 astrocytoma. Tumor enhancement, intratumoral-necrosis like presentation, larger peritumoral edema, and more explicit tumor margins were frequently observed in histological WHO Grade 4 astrocytoma. Additionally, molecular WHO Grade 4 astrocytoma showed a tendency for relatively longer overall survival, while a statistical significance was not reached (47 vs. 25 months, p = 0.22). TP53, CDK6, and PIK3CA alteration was commonly observed, while PIK3R1 (p = 0.033), Notch1 (p = 0.027), and Mycn (p = 0.027) alterations may affect the overall survival of molecular WHO Grade 4 astrocytomas.
Our study scrutinized IDH-mutant, Grade 4 astrocytoma. Therefore, further classification should be considered as the prognosis varied between histological and molecular WHO Grade 4 astrocytomas. Notably, therapies aiming at PIK3R1, Notch 1, and Mycn may be beneficial.