Primary intracranial dedifferentiated liposarcoma: An extremely rare site with unusual histopathological findings.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Neuropathology Pub Date : 2024-11-04 DOI:10.1111/neup.13012
Sumanta Das, Rakesh Kumar Gupta, Jayati Sarangi, Priti Jain, Ramana Gogi, Rana Patir, Sunita Ahlawat
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引用次数: 0

Abstract

Primary intracranial sarcomas constitute a rare group of tumors, with the most common types described in the literature being chondrosarcoma and fibrosarcoma. Dedifferentiated liposarcoma (DDLS) is a high-grade sarcoma that sometimes metastasizes to the brain. However, a primary intracranial DDLS is exceedingly rare. A 45-year-old patient from the Middle East came to India for treatment. His magnetic resonance imaging (MRI) scans revealed a space-occupying lesion at the level of the lateral ventricle T2/fluid attenuated inversion recovery hyperintensity with peripheral edema. A T1 perfusion map showed high relative cerebral blood volume values in the peripheral part, suggesting a high-grade neoplasm. Gross total resection was performed, and histopathology showed a high-grade tumor composed of sheets of pleomorphic lipoblasts and epithelioid tumor cells arranged in nests and cords. Immunohistochemistry showed diffuse immunopositivity for MDM2, CDK4, and p16, while GFAP and OLIG2 were negative. Fluorescence in situ hybridization showed MDM2 amplification. Final diagnosis of DDLS was rendered. The patient had no systemic lesions elsewhere on positron emission tomography computed tomography scan.

原发性颅内低分化脂肪肉瘤:组织病理学发现极为罕见的部位。
原发性颅内肉瘤是一类罕见的肿瘤,文献中最常见的类型是软骨肉瘤和纤维肉瘤。未分化脂肪肉瘤(DDLS)是一种高级别肉瘤,有时会转移到脑部。然而,原发性颅内 DDLS 却极为罕见。一名 45 岁的患者从中东来到印度接受治疗。他的磁共振成像(MRI)扫描显示,侧脑室T2/流体衰减反转恢复高密度伴周围水肿的占位性病变。T1 灌注图显示外周部分的相对脑血容量值较高,提示为高级别肿瘤。进行了大体全切除,组织病理学显示,这是一个由多形性脂母细胞和上皮样肿瘤细胞组成的高级别肿瘤,这些细胞呈巢状和条索状排列。免疫组化显示,MDM2、CDK4和p16呈弥漫性免疫阳性,而GFAP和OLIG2呈阴性。荧光原位杂交显示 MDM2 扩增。最终诊断为 DDLS。正电子发射计算机断层扫描显示,患者其他部位没有系统性病变。
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
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.
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