CAJ | 학술논문

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的临床病理、免疫表型特征及其与EB病毒的关系.方法分析15例经病理确诊的PCNSL患者临床资料,归纳其临床病理和影像学特征.采用EnVision免疫组织化学法及EB病毒寡核苷酸探针(EBER)原位杂交,观察其免疫表型和EB病毒感染.结果 PCNSL患者以颅内高压(9例)、肢体偏瘫或无力(5例)为主要表现.组织学特点为瘤细胞形态较单一,弥漫分布,或围绕血管呈袖套状浸润,伴灶性坏死及出血.免疫组织化学染色结果显示,15例均为弥漫性大B细胞淋巴瘤(DLBCL),5例为生发中心型,10例为非生发中心型.EBER原位杂交显示仅1例(6.7％)出现阳性.平均生存期16个月(3～32个月).结论 PCNSL术前难以诊断,需依赖病理检查确诊.本组免疫表型均为B细胞源性,且与EB病毒呈低相关性,预后差.
목적 탐토원발성중추신경계통림파류(PCNSL)적림상병리、면역표형특정급기여EB병독적관계.방법 분석15례경병리학진적PCNSL환자림상자료,귀납기림상병리화영상학특정.채용EnVision면역조직화학법급EB병독과핵감산탐침(EBER)원위잡교,관찰기면역표형화EB병독감염.결과 PCNSL환자이로내고압(9례)、지체편탄혹무력(5례)위주요표현.조직학특점위류세포형태교단일,미만분포,혹위요혈관정수투상침윤,반조성배사급출혈.면역조직화학염색결과현시,15례균위미만성대B세포림파류(DLBCL),5례위생발중심형,10례위비생발중심형.EBER원위잡교현시부1례(6.7％)출현양성.평균생존기16개월(3～32개월).결론 PCNSL술전난이진단,수의뢰병리검사학진.본조면역표형균위B세포원성,차여EB병독정저상관성,예후차.
Objective To investigate the clinicopathological features,immunophenotypes and Epstein-Barr (EB) virus infection of primary central nervous system lymphoma (PCNSL).Methods The clinicopathologic and radiologic features of 15 cases of PCNSL were analyzed retrospectively.All samples were stained by EnVision immunohistochemistry and in situ hybridization was performed for detection of EB virus encoded small nuclear RNAs (EBER).Results Increased intracranial pressure (9 cases),hemiparalysis or weakness (5 cases) were the main clinical presentations of PCNSL without specific imaging findings.Morphologically,tumor cells were uniform and diffuse infiltration,or showed a characteristic perivascular cuffs pattern,with areas of necrosis and hemorrhage.Fifteen cases of PCNSLs were all dignosed as having diffuse large B-cell lymphomas (DLBCLs),5 having germinal center subtype and 10 having nongerminal center subtype based on the immunohistochemical study.Only 1/15 (6.7％) cases showed positive signal in EBER in situ hybridization.Mean overall survival was 16 months (range,3-32 months).Conclusion PCNSL is difficult to diagnose preoperatively.The final diagnosis is confirmed on the histopathological and immunohistochemical examinations.All of PCNSLs in this group are of B-cell origin and may be poorly associated with Epstein-Barr virus.The prognosis is poor.