摘要
目的 提高中枢神经系统淋巴瘤样肉芽肿病(central nervous system lymphomatoid granulomatosis,CNSLyG)的临床病理认识。方法 回顾性分析4例CNS-LyG患者的病例资料,包括临床表现、病理形态、免疫组化及原位杂交,并结合文献分析。结果 CNS-LyG临床表现因病变部位及损害程度不同而异,血清学无特征,CT或MRI提示病变部位呈无特征性斑片状或条索状增强。病理形态表现为脑组织中增生的毛细血管周围有数量不等轻度幼稚淋巴细胞样细胞,围绕血管呈片状分布。免疫标记常为多克隆性。EB病毒编码的小RNA(epstein-barr virus-encoded small RNA,EBER)检测结果阴性,治疗以手术为主,辅以免疫疗法和(或)放化疗。预后与病变损害部位、大小及其分级有关。结论 CNS-LyG罕见,其诊断需结合临床表现、病理形态、免疫组化、原位杂交等综合分析。当异型细胞免疫标记单一表达时,提示恶性病变可能,需进一步完善免疫组化及分子检测,CNS-LyG预后与病理分级有关。
Objective To enhance the clinicopathological understanding of central nervous system lymphomatoid granulomatosis(CNS-LyG).Methods Clinical data of 4 patients with CNS-LyG were analyzed retrospectively,including clinical manifestations,pathological morphology,immunohistochemistry,and in situ hybridization,supplemented by a literature review.Results The clinical manifestations of CNS-LyG varied depending on the lesion location and extent of damage,with no characteristic serological findings.CT or MRI revealed non-characteristic patchy or strip-like enhancement at the lesion site.Pathologically,it was characterized by the presence of varying numbers of mildly immature lymphocyte-like cells proliferating around capillaries in brain tissue,forming sheet-like distributions.Immunohistochemical markers were often polyclonal.The Epstein-Barr virus-encoded RNA(EBER)test results were negative.Treatment primarily involved surgery,supplemented by immunotherapy and/or radiotherapy/chemotherapy.Prognosis was associated with the location,size,and grade of the lesion.Conclusions CNS-LyG is rare,and its diagnosis requires a comprehensive analysis combining clinical manifestations,pathological morphology,immunohistochemistry,and in-situ hybridization.When atypical cells show single immunohistochemical marker expression,it suggests the possibility of malignant lesions,necessitating further immunohistochemical and molecular testing.The prognosis of CNS-LyG is related to its pathological grading.
作者
王晓露
王圣超
王晓阳
叶雅莉
宗嘉雯
岳梨
陈琳
王卓才
WANG Xiaolu;WANG Shengchao;WANG Xiaoyang;YE Yali;ZONG Jiawen;YUE Li;CHEN Lin;WANG Zuocai(Department of Pathology,General Hospital of Southern Theater Command,Guangzhou 510010,China;School of Stomatology,Jinan University,Guangzhou 510632,China)
出处
《中国微侵袭神经外科杂志》
2025年第11期674-678,共5页
Chinese Journal of Minimally Invasive Neurosurgery
关键词
中枢神经系统淋巴瘤样肉芽肿病
临床病理
免疫组化
central nervous system lymphomatoid granulomatosis
clinicopathology
immunohistochemistry
