颅内亲血管性B-细胞淋巴瘤一例报告并文献复习

Angiotropic B - cell Lymphomas in Central Nervous System: A Case Report and Literature Review

原发性中枢系统淋巴瘤(PCNSL)中B细胞淋巴瘤占绝大多数,主要表现为局灶病变。亲血管性淋巴瘤(Angiotropic Lymphoma)又称血管内淋巴瘤(Intravascular Lymphoma),是淋巴瘤的特殊类型,原发性中枢神经系统(CNS)亲血管性淋巴瘤罕见,组织学检查主要为亲血管性B-细胞淋巴瘤,国内外文献仅见个案报告。本文报告我们收治的1例颅内亲血管性B-细胞淋巴瘤。方法:报告1例颅内亲血管性B-细胞淋巴瘤,对其临床、病理、影像学特点和治疗结合复习相关文献进行讨论。结果:原发性CNS亲血管性B-细胞淋巴瘤罕见,临床表现缺乏特异性。其病理学特征是病灶及其周围脑血管明显扩张,肿瘤细胞积聚于小血管内、血管周围间隙和毛细血管内,导致血管堵塞而形成局部小梗死灶。异型的淋巴细胞免疫组化标记LCA、CD20和CD79阳性,最后确诊为亲血管性B-细胞淋巴瘤。结论:原发性CNS亲血管性B-细胞淋巴瘤临床表现多样,确诊主要依据组织病理学检查,及早确诊和综合治疗是提高治疗效果的关键。

BACKGROND & OBJECTIVE: The most of primary central nervous system lymphomas(PCNSL) are B-cell lymphomas. Angiotropic lymphoma, also known as intravascular lymphoma, is a special subtype of lymphoma and rarely presented primary in central nervous system. Histopathologically, most of this kind tumors were B-cell lymphomas. In this paper we report a case of primary central nervous system angiotropic B-cell lymphoma. METH-ODS: A female patient with angiotropic B-cell lymphoma in central nervous system was reported, and its clinical manifesta-tion, histopathologic characteristics, imaging characteristics and treatment were discussed. Related literatures were re-viewed. RESULTS: The primary central nervous system angiotropic B-cell lymphomas are rarely. The histopathologi-cal characteristics were obviously cerebrovascular dilation in tumor, accumulation of tumor cells within the capillary and in the small and medium size vessels or at peri-vascu-larspacethatleadstovascularocclusionanddisseminat-ed small infarcts. The immunophenotype of the tu-mor cells in vascular is LCA、CD20、CD79 positive,that helps confirming diagnosis of angiotropic B-cell lymphomas. CONCLUSION: The primary cen-tral nervous system angiotropic B-cell lymphomas have various clinical manifestations. Its diagnosis depends on histopathologic examination. The early diagnosis and mutimudal-ity therapy play an important role to improve the prognosis.