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圆锥马尾区域具有毛细胞特征的高级别星形细胞瘤:世界卫生组织新诊断分类中的罕见表现。病例报告

High-grade astrocytoma with piloid features in the conus medullaris: a rare presentation of a new World Health Organization diagnosis. Illustrative case.

作者信息

Ndirangu Brandy, Neill Stewart, Cory Adamson David

机构信息

Emory University School of Medicine, Atlanta, Georgia.

Department of Pathology, Emory University School of Medicine, Atlanta, Georgia.

出版信息

J Neurosurg Case Lessons. 2024 Oct 28;8(18). doi: 10.3171/CASE24390.

DOI:10.3171/CASE24390
PMID:39467315
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11525760/
Abstract

BACKGROUND

Neuroepithelial tumors with histological features of pilocytic astrocytoma (PA), increased mitotic activity with high-grade features of microvascular proliferation, and palisading necrosis have often been designated as anaplastic PAs. High-grade astrocytoma with piloid features (HGAP) was recently classified as its own entity by the World Health Organization (WHO) in 2021, after being grouped under anaplastic PA. This rare tumor typically arises in the cerebellum. The authors present the case of a patient with HGAP in the conus medullaris, a rare occurrence, with only 17 reported HGAP cases in other parts of the spinal cord.

OBSERVATIONS

An 83-year-old female patient presented with progressive lower-extremity weakness over 2 weeks. Subsequent imaging revealed an intramedullary spinal cord mass near the conus. Histopathological analysis confirmed a neuroepithelial tumor. Chromosomal microarray revealed the diagnosis of HGAP, an exceedingly unusual tumor to be found near the conus. The tumor characteristics showed uniformly low cellularity and low proliferation of medium-sized ovoid cells with elongated nuclei, embedded in a loose pilocytic matrix.

LESSONS

This report presents the case of a patient with HGAP in the conus medullaris, a new WHO diagnosis that needs to be included in the differential diagnosis for conus masses. https://thejns.org/doi/10.3171/CASE24390.

摘要

背景

具有毛细胞型星形细胞瘤(PA)组织学特征、有微血管增殖和栅栏状坏死等高级别特征的有丝分裂活性增加的神经上皮肿瘤,常被指定为间变性PA。具有毛细胞样特征的高级别星形细胞瘤(HGAP)在被归类为间变性PA后,于2021年被世界卫生组织(WHO)列为一种独立的实体。这种罕见肿瘤通常发生在小脑。作者报告了1例脊髓圆锥HGAP患者,这种情况很罕见,在脊髓其他部位仅有17例HGAP病例报告。

观察结果

一名83岁女性患者在2周内出现进行性下肢无力。随后的影像学检查显示脊髓圆锥附近有髓内脊髓肿块。组织病理学分析证实为神经上皮肿瘤。染色体微阵列分析确诊为HGAP,这是一种在脊髓圆锥附近发现的极其罕见的肿瘤。肿瘤特征显示细胞密度均匀较低,中等大小的卵圆形细胞增殖较低,细胞核细长,嵌入疏松的毛细胞基质中。

经验教训

本报告介绍了1例脊髓圆锥HGAP患者,这是WHO的一种新诊断,在脊髓圆锥肿块的鉴别诊断中需要考虑到。https://thejns.org/doi/10.3171/CASE24390。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/a1d485bfacb5/CASE24390_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/c741ad951b3d/CASE24390_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/8d836311c6a2/CASE24390_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/a1d485bfacb5/CASE24390_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/c741ad951b3d/CASE24390_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/8d836311c6a2/CASE24390_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0490/11525760/a1d485bfacb5/CASE24390_figure_3.jpg

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