原发性中枢神经系统弥漫性大B细胞性淋巴瘤围血管生长机制的探讨

Mechanism of angiotropism in primary central nervous system diffuse large B cell lymphoma

目的探讨原发性中枢神经系统弥漫性大B细胞性淋巴瘤(PCNS-DLBCL)围血管生长的机制。方法对22例PCNS-DLBCL进行临床、放射和病理观察及随访,免疫组化EnVision法行XBP1、CD44和IL-4表达分析,以17例淋巴结弥漫性大B细胞性淋巴瘤和10例胶质母细胞瘤为对照。结果 22例PCNS-DLBCL年龄41～69岁,平均年龄56岁,男女之比为1.4∶1。CT多表现为占位性病变;MRI显示T1加权相低信号,T2加权相等或稍高信号,增强扫描有不均匀强化。镜检示肿瘤细胞大多围绕血管生长形成袖套样外观,实性区域仍伴有围血管生长特点。免疫组化示围血管生长的肿瘤细胞XBP1、IL-4和CD44强(+),表达率分别为95.5%、86.3%和77.3%,GFAP和CD34(-),与对照组淋巴结弥漫性大B细胞性淋巴瘤和胶质母细胞瘤比较差异极显著(P<0.01)。结论 PCNS-DLBCL具有特征性的临床病理特征,肿瘤细胞围血管生长显示独特的形态特点,与XBP1、CD44和IL-4的表达增高关系密切。

Objective To explore mechanism of angiotropism in primary central nervous system diffuse large B cell lymphoma（PCNS-DLBCL）.Methods Clinical,radiologic and pathologic features,therapy and follow-up were studied in 22 cases of PCNS-DLBCL.Immunohistochemical staining was performed by EnVision method using the related molecular markers such as XBP1,IL4 and CD44.17 cases of diffuse large B cell lymphoma in lymph nodes and 10 cases of glioblastoma were studied as control.Results All tumors were primarily located in the central nervous system.The male to female ratio was 1.4∶1.The patient＇s age were 42 to 69 years（median 56）.The computer tomography showed a mass and MRI showed the tumor in T1 weighted images to be of lower signal intensity and on T2 weighted images to be homo or higher signal intensity with an uneven contrast enhancement.Most tumor cells were accumulated within the perivascular space,and even in more solid pattern there were still some accumulations in the perivascular space.The tumor cells of PCNS-DLBCL were positve for XBP1,IL4 and CD44（the positive rate was 95.5%,86.3% and 77.3%,respectively）,and negative for GFAP and CD34.There were significant differences in the expression of XBP1,IL4 and CD44 between PCNS-DLBCL and control groups（P0.01）.Conclusions PCNS-DLBCL has specific clinical pathological features,with morphologic angiotropism.The tumor cells surrounding blood vessels express XBP1,IL4 and CD44,which may be related to the growth pattern of tumor cells in perivascular space.