原发性中枢神经系统弥漫性大B细胞淋巴瘤临床病理诊断并相关文献复习（附4例）

目的:探讨原发性中枢神经系统弥漫性大B细胞淋巴瘤(primary central nervous system diffuse large B cell lymphoma,PCNS-DLBCL)的临床病理学特点。方法:对4例原发性中枢神经系统弥漫性大B细胞淋巴瘤的临床表现、大体、组织学特征及免疫组化等进行观察,并复习相关文献。结果:患者男性1例,女性3例,平均年龄56.25岁;病变均位于幕上,临床表现多样,MRI表现具有一定特异性。肿瘤细胞呈圆形、卵圆形,中等偏大,可见核仁,核分裂象多见,可见病理性核分裂,有坏死,肿瘤细胞亲血管生长形成淋巴细胞血管套。免疫组化示肿瘤细胞表达B细胞相关抗原,4例均表达CD20(+）,Ki-67平均增殖指数为 70%。结论:原发性中枢神经系统弥漫性大B细胞淋巴瘤是一种罕见恶性肿瘤,预后差,诊断应结合临床资料及影像学检查,并需与多种良、恶性肿瘤相鉴别。目前治疗首选大剂量甲氨蝶呤为基础联合利妥昔单抗的化疗方案,辅以全脑放疗、自体干细胞移植等巩固治疗。

Primary central nervous system diffuse large B-cell lymphoma:A study of 4 cases and review of literature

Objective:To analyze the clinical and pathologic features in primary central nervous system diffuse large B-cell lymphoma.Methods:4 cases of primary central nervous system diffuse large B-cell lymphoma were studied with histology and immunohistochemical staining,and it's clinical and pathological findings were further analyzed with review of literature.Results:Among 4 cases,one was male and 3 were females and mean age was 56.25 years old.The 4 cases were all located at the supratentorial brain.These tumor cells were medium to large,with nucleoli and mitosis,also have pathological mitosis and necrosis.The tumor cells grow into lymphocytic vascular sheath.Immunophenotypically,these tumor cells were positive for CD20,with a high Ki-67 proliferation index(70%).Conclusion:Primary central nervous system diffuse large B-cell lymphoma is a rare malignant neoplasm.The clinical and radiologic findings must be known for differential diagnosis from other tumors.Primary central nervous system diffuse large B-cell lymphoma has a worse prognosis.Stereotactic biopsy is the gold standard of diagnosis.Treating with high dose of methotrexate combining with rituximab is the preferred treatment for this tumor,followed by whole-brain radio therapy and autologous stem cell transplantation.