Abstract:
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is considered one of the low-grade neuroepithelial tumors, as per the World Health Organization 2021 classification of brain tumors. First described in 2016, these morphologically variable tumors are characterized by oligodendroglioma-like cellular components, infiltrative growth patterns, and cluster of differentiation 34 immunopositivity. A literature search of the PubMed/MEDLINE, SCOPUS, ScienceDirect, and COCHRANE databases (from inception to 20th June 2022) was carried out to identify relevant studies. To identify additional studies, we performed a recursive search of the bibliographies of the selected articles and published systematic reviews on this topic. The search yielded a total of 64 results. After removing duplicates, 26 articles were eligible for the review. The diagnostic criteria for these glioneuronal variants, representing a broad neuropathological spectrum, are not distinct and hence impede proper diagnosis and prognosis. Frequent genetic abnormalities involving mitogen-activated protein kinase pathway constituents, such as B-Raf proto-oncogene or fibroblast growth receptor 2/3, are harbored by PLNTYs. Recent advances in molecular diagnostics have resulted in more accurate tumor classification systems, based on gene expression profiles and DNA methylation patterns. Gross total resection seems curative, with a low recurrence rate. Malignant transformation is rare; however, adjuvant radiation therapy and chemotherapy may be beneficial in selected cases.
摘要:
年轻人的多形性低度神经上皮肿瘤(PLNTY)被认为是低度神经上皮肿瘤之一,根据世界卫生组织2021年的脑肿瘤分类。在2016年首次描述,这些形态可变的肿瘤的特征是少突胶质细胞瘤样细胞成分,渗透生长模式,和分化群34免疫阳性。PubMed/MEDLINE的文献检索,Scopus,ScienceDirect,和COCHRANE数据库(从开始到2022年6月20日)进行了识别相关研究。为了确定更多的研究,我们对所选文章的参考书目进行了递归搜索,并发表了有关该主题的系统评论。搜索总共产生了64个结果。删除重复项后,26篇文章符合审查条件。这些神经胶质神经元变异的诊断标准,代表广泛的神经病理学谱,不明显,因此阻碍了正确的诊断和预后。涉及丝裂原活化蛋白激酶途径成分的频繁遗传异常,例如B-Raf原癌基因或成纤维细胞生长受体2/3,被PLNTY携带。分子诊断的最新进展导致了更准确的肿瘤分类系统,基于基因表达谱和DNA甲基化模式。大体全切除似乎治愈了,复发率低。恶性转化是罕见的;然而,辅助放疗和化疗在某些病例中可能是有益的。