Pediatric and elderly polymorphous low-grade neuroepithelial tumor of the young: Typical and unusual case reports and literature review.

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Neuropathology Pub Date : 2023-08-01 DOI:10.1111/neup.12889

Takuya Furuta, Mayuko Moritsubo, Hiroko Muta, Hotetsu Shimamoto, Koichi Ohshima, Yasuo Sugita

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引用次数: 2

Abstract

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY), one of the pediatric-type diffuse low-grade gliomas, is characterized by a diffuse infiltrating pattern of oligodendroglioma-like tumor cells showing CD34 positivity and harbors mitogen-activated protein kinase (MAPK) alteration, such as vRAF murine sarcoma viral oncogene homolog B1 (BRAF) p.V600E or fibroblast growth factor fusion genetically. It occurs mainly in pediatric and adolescents with seizures due to the dominant location of the temporal lobe. However, there have been a few cases of PLNTY in adult patients, suggesting the wide range of this tumor spectrum. Here, we describe two cases of PLNTY, one in a 14-year-old female and the other in a 66-year-old female. The pediatric tumor showed typical clinical course and histopathology with BRAF p.V600E mutation, whereas the elderly tumor was unusual because of non-epileptic onset clinically and ependymal differentiation histopathologically harboring KIAA1549-BRAF fusion. There might be unusual but possible PLNTY, as in our elderly case. We also compared typical pediatric and unusual elderly tumors by reviewing the literature.

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儿童和老年人多形低级别神经上皮肿瘤:典型和不寻常的病例报告和文献复习。

多形性低级别神经上皮肿瘤(PLNTY)是儿童型弥漫性低级别神经胶质瘤之一，其特征是少突胶质细胞样肿瘤细胞呈弥漫性浸润模式，CD34阳性，含有丝裂原活化蛋白激酶(MAPK)改变，如vRAF鼠肉瘤病毒癌基因同源物B1 (BRAF) p.V600E或成纤维细胞生长因子基因融合。它主要发生在儿童和青少年癫痫发作，由于颞叶的主要位置。然而，在成年患者中也有少数PLNTY病例，这表明这种肿瘤的范围很广。在这里，我们描述了两个PLNTY病例，一个发生在14岁的女性，另一个发生在66岁的女性。儿童肿瘤表现为典型的BRAF p.V600E突变的临床病程和组织病理学，而老年肿瘤则因临床非癫痫性发病和室管膜分化组织病理学上存在KIAA1549-BRAF融合而不常见。可能是不寻常的，但也可能是大量的，就像我们这位老人的情况一样。我们也通过回顾文献比较了典型的儿科和不寻常的老年肿瘤。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuropathology 医学-病理学

CiteScore

4.10

自引率

4.30%

发文量

105

审稿时长

6-12 weeks

期刊介绍： Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.