Imai Hiroshi, Kajimoto Kazuyoshi, Taniwaki Masashi, Miura Ikuo, Hatta Yoshihiro, Hashizume Yoshio, Watanabe Masatoshi, Shiraishi Taizo, Nakamura Shigeo
Department of Pathology, Mie University School of Medicine, Tsu, Mie, Japan.
Pathol Int. 2004 Apr;54(4):231-6. doi: 10.1111/j.1440-1827.2004.01613.x.
We have encountered five cases of intravascular large B-cell lymphoma (IVL) presenting with central nervous system (CNS) mass lesions during their clinical course. The age of the patients ranged from 50 to 74 years and three patients were male. All of these cases histopathologically showed typical intravascular localization of the neoplastic cells in the initial biopsy specimens obtained from sites other than the CNS. Despite multiagent chemotherapy, patients suffered from single or multiple CNS mass lesions 5-44 months after the initial diagnosis of IVL, except for one case in which IVL and the CNS mass lesion were diagnosed at the same time. The subsequent biopsy and autopsy specimens obtained from the CNS mass lesions revealed diffuse infiltration of the tumor cells with perivascular spreading, but minimal or no intravascular components. Immunohistochemical analysis of intravascular tumor cells and CNS mass lesions revealed expression of CD20, CD79a, bcl-2 and negative for CD3e and Epstein-Barr virus encoded RNA. The overall features of the CNS mass lesions were very similar to or indistinguishable from those of the primary CNS lymphomas. This implies that CNS mass lesions in the IVL cases can be correctly diagnosed only by careful attention to clinical and pathological findings. Moreover, there is the possibility that some cases previously diagnosed as primary CNS lymphomas may have include IVL cases. Further investigation is needed to explore this unusual phenomenon.
我们遇到了5例血管内大B细胞淋巴瘤（IVL），这些病例在临床过程中出现中枢神经系统（CNS）肿块病变。患者年龄在50至74岁之间，3例为男性。所有这些病例在从CNS以外部位获取的初始活检标本中，组织病理学均显示肿瘤细胞典型的血管内定位。尽管进行了多药化疗，但患者在IVL初始诊断后5至44个月出现单个或多个CNS肿块病变，其中1例IVL与CNS肿块病变同时被诊断。随后从CNS肿块病变获取的活检和尸检标本显示肿瘤细胞弥漫浸润并伴有血管周围扩散，但血管内成分极少或无。对血管内肿瘤细胞和CNS肿块病变进行免疫组织化学分析显示，CD20、CD79a、bcl-2表达阳性，CD3e和爱泼斯坦-巴尔病毒编码RNA表达阴性。CNS肿块病变的总体特征与原发性CNS淋巴瘤非常相似或难以区分。这意味着IVL病例中的CNS肿块病变只有通过仔细关注临床和病理结果才能正确诊断。此外，有可能一些先前被诊断为原发性CNS淋巴瘤的病例可能包括IVL病例。需要进一步研究来探索这种不寻常的现象。
