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骨肉瘤:一例具有独特特征的罕见病例报告。

Olıgosarcoma: A Rare Case Report Wıth Dıstınct Features.

作者信息

Calim Gurbuz Begum, Soylemez Akkurt Tuce, Kusku Cabuk Fatmagul, Sencan Fahir, Tugcu Bekir, Ersen Danyeli Ayca

机构信息

Pathology Department, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey.

Neurosurgery Department, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey.

出版信息

Int J Surg Pathol. 2025 Jun;33(4):964-969. doi: 10.1177/10668969241291891. Epub 2024 Nov 20.

DOI:10.1177/10668969241291891
PMID:39563510
Abstract

Oligosarcoma is a recently identified entity characterized by sarcomatous changes originating from oligodendroglioma. As of our current understanding, sarcomatous components are infrequent in glial tumors. The World Health Organization (WHO) classification describes sarcomatous features as a rare pattern in grade 3 oligodendrogliomas. In this report, we present a 42-year-old man diagnosed with oligosarcoma. The patient initially presented with a lesion in the right parietotemporal area 9 years ago, and the pathological diagnosis was oligodendroglioma. Nine years later, a recurrent lesion in the same area was observed. Histomorphological evaluation of the recurrent lesion revealed distinct glial and sarcomatous components. The diagnosis of oligosarcoma was made based on histologic assessment; however, additional histochemical (reticulin-rich sarcomatous area), immunohistochemical, and molecular evaluations were also conducted. Immunohistochemical marker expression patterns in oligosarcoma have been reported variably in the literature. In our patient, the sarcomatous component exhibited p53 and OLIG2 immunohistochemical expression. Molecular analysis revealed IDH and TERT mutations, as well as 1p/19q and CDKN2A deletions.

摘要

少突肉瘤是一种最近发现的实体瘤,其特征是起源于少突胶质细胞瘤的肉瘤样改变。就我们目前的认识而言,肉瘤样成分在胶质肿瘤中并不常见。世界卫生组织(WHO)分类将肉瘤样特征描述为3级少突胶质细胞瘤中的一种罕见模式。在本报告中,我们介绍了一名42岁被诊断为少突肉瘤的男性患者。该患者最初于9年前在右侧顶颞区出现一个病灶,病理诊断为少突胶质细胞瘤。9年后,在同一区域观察到复发病灶。对复发病灶的组织形态学评估显示出明显的胶质和肉瘤样成分。少突肉瘤的诊断基于组织学评估;然而,还进行了额外的组织化学(富含网硬蛋白的肉瘤样区域)、免疫组织化学和分子评估。少突肉瘤中免疫组织化学标志物的表达模式在文献中的报道各不相同。在我们的患者中,肉瘤样成分表现出p53和OLIG2免疫组织化学表达。分子分析显示存在异柠檬酸脱氢酶(IDH)和端粒酶逆转录酶(TERT)突变,以及1p/19q和细胞周期蛋白依赖性激酶2A(CDKN2A)缺失。

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