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原发性颅内浆母细胞淋巴瘤伴硬脊膜外髓外转移:一例报告

Primary intracranial plasmablastic lymphoma with intradural extramedullary metastasis: a case report.

作者信息

Jiang Zhuoru, Zhu Zhengyang, Tao Zhennan, Niu Fengnan, Zhang Xin, Zhang Bing

机构信息

Department of Radiology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, 210009, China.

Institute of Medical Imaging and Artificial Intelligence, Nanjing University, Nanjing, 210093, China.

出版信息

BMC Neurol. 2025 Sep 29;25(1):394. doi: 10.1186/s12883-025-04409-9.

DOI:10.1186/s12883-025-04409-9
PMID:41023644
Abstract

BACKGROUND

Primary central nervous system plasmablastic lymphoma (PCNSPBL) represents an exceptionally rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL), characterized by its distinct immunophenotypic profile and predilection for immunocompromised individuals. Accurate preoperative diagnosis remains challenging due to the nonspecific radiological features observed on conventional magnetic resonance imaging (MRI), necessitating comprehensive histopathological evaluation for definitive diagnosis. While intracranial involvement of PBL has been documented in the literature, intraspinal metastasis of this malignancy has not been previously reported. This study presents a novel case of PCNSPBL with concurrent spinal cord metastasis in an elderly male patient, with a detailed analysis of advanced neuroimaging characteristics that may aid in the diagnostic evaluation of this rare entity.

CASE PRESENTATION

A 52-year-old male patient presented to our institution with left-sided limb weakness. Laboratory investigations revealed positive Epstein-Barr virus-encoded small RNA (EBER). Conventional MRI demonstrated a mass lesion with a clear anatomical relationship to the inferior horn of the right lateral ventricle, presenting as compression and stenosis of the right ventricle. Due to the mass effect, the patient underwent surgical resection of the right temporal lobe lesion, with histopathological examination confirming the diagnosis of plasmablastic lymphoma.

CONCLUSIONS

This report represents the first documented case of PCNSPBL with intradural extramedullary metastasis, underscoring the diagnostic challenges associated with this rare malignancy. Advanced neuroimaging techniques, including functional MRI, serve as valuable complements to conventional MRI in the diagnostic workup. Early and accurate diagnosis of PBL is critical for timely intervention, and further studies are warranted to establish standardized imaging protocols for this rare entity.

摘要

背景

原发性中枢神经系统浆母细胞性淋巴瘤(PCNSPBL)是弥漫性大B细胞淋巴瘤(DLBCL)中一种极为罕见且侵袭性强的亚型,其特征在于独特的免疫表型特征以及对免疫功能低下个体的偏好。由于在传统磁共振成像(MRI)上观察到的非特异性放射学特征,准确的术前诊断仍然具有挑战性,因此需要进行全面的组织病理学评估以明确诊断。虽然文献中已记录了PBL的颅内受累情况,但此前尚未报道过这种恶性肿瘤的脊髓转移。本研究报告了一例老年男性患者并发脊髓转移的PCNSPBL新病例,并对可能有助于该罕见实体诊断评估的先进神经影像学特征进行了详细分析。

病例介绍

一名52岁男性患者因左侧肢体无力前来我院就诊。实验室检查显示爱泼斯坦-巴尔病毒编码的小RNA(EBER)呈阳性。传统MRI显示一个与右侧脑室下角有明确解剖关系的肿块病变,表现为右侧脑室受压和狭窄。由于肿块效应,患者接受了右颞叶病变的手术切除,组织病理学检查确诊为浆母细胞性淋巴瘤。

结论

本报告是首例有硬脊膜外髓外转移的PCNSPBL病例,强调了与这种罕见恶性肿瘤相关的诊断挑战。包括功能MRI在内的先进神经影像学技术在诊断检查中是对传统MRI的有价值补充。PBL的早期准确诊断对于及时干预至关重要,有必要进一步开展研究以建立针对这种罕见实体的标准化成像方案。

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