摘要：

目的 报告1 例具有溶骨性表现、向颅内和眶内侵袭性生长的B 小细胞淋巴瘤/慢性淋巴细胞白血病患者，结合文献对其临床表现、影像学特点、组织学形态和免疫组织化学表型、诊断与治疗策略进行分析。方法与结果 女性患者，60 岁，临床主要表现为左侧眼眶肿胀伴间断性头痛。头部MRI显示，左侧额颞叶、左侧蝶骨大翼、左侧蝶窦外侧壁、左侧眶外侧壁和上壁占位性病变；三维重建CT 显示，左侧额骨、颞骨、蝶骨骨质广泛性破坏。于全身麻醉下行肿瘤切除术。组织学形态观察，肿瘤细胞呈弥漫性分布，胞核小而圆、染色质凝集深染、偶见核仁，胞质极少。免疫组织化学染色，肿瘤细胞胞膜CD5 呈弥漫性强阳性，CD20、CD43 阳性，CD23 部分阳性，CD138 小灶性阳性，CD38 散在阳性，黑色素瘤相关抗原突变型MUM1 个别阳性，胞膜和胞质上皮膜抗原阳性，胞质免疫球蛋白κ链阳性；而细胞周期蛋白D1、CD10、CD56、Bcl-6、胶质纤维酸性蛋白、突触素和免疫球蛋白λ链均呈阴性；Ki-67抗原标记指数约70%。最终病理诊断为B 小细胞淋巴瘤/慢性淋巴细胞白血病。术后辅以药物化疗。随访6 个月仍生存且生活质量满意。结论 中枢神经系统淋巴瘤临床表现多样、影像学表现不典型，明确诊断依靠组织病理学检查，B 小细胞淋巴瘤/慢性淋巴细胞白血病应注意与中枢神经系统转移瘤、其他原发性中枢神经系统肿瘤和其他血液系统疾病相鉴别，治疗方面于神经导航下大部分切除肿瘤，术后辅以药物化疗和放射治疗可取得较好疗效。

关键词: 白血病, 淋巴细胞, 慢性, B 细胞, 眼眶, 肿瘤侵润, 免疫组织化学, 病理学

Abstract:

Objective  To report a case of B-small lymphocytic lymphoma (SLL)/chronic lymphocytic leukemia (CLL) with osteolytic performance invading the intracranial and orbital part, and to analyze the clinical manifestations, imaging features, histological patterns and immunohistochemical phenotypes, diagnosis and treatment strategies of this disease combined with review of literatures.  Methods and Results  A 60-year-old female presented with left orbital swelling with intermittent headache. Head MRI showed space-occupying lesions invading left frontotemporal lobe, left greater wing of sphenoid bone, left lateral wall of sphenoid sinus, left lateral and upper orbital wall. Three-dimensional reconstructed CT showed extensive bone destruction in left frontal, temporal and sphenoid bone. The patient underwent tumor resection under general anesthesia. Histologically, the tumor cells were diffusely distributed. The nuclei were small, round and hyperchromatic, with sparse nucleoli and cytoplasm. The membrane of tumor cells were diffusely positive for CD5, positive for CD20 and CD43, partially positive for CD23, focally positive for CD138, sparsely positive for CD38 and sporadically positive for MUM1. The membrane and cytoplasm of tumor cells were positive for epithelial membrane antigen (EMA). The cytoplasm was positive for immunoglobulin κ-chain. Cyclin D1, CD10, CD56, Bcl-6, glial fibrillary acidic protein (GFAP), synaptophysin (Syn) and immunoglobulin λ-chain were negative. Ki-67 labeling index was about 70% . Final pathological diagnosis was B-SLL/CLL. The patient was treated by postoperative chemotherapy, and the 6-month follow-up showed a fine survival.  Conclusions  The clinical manifestations of central nervous system (CNS) lymphomas are various, and the imaging features are atypical. A definite diagnosis depends on histopathological diagnosis. B-SLL/CLL should be differentiated from CNS metastatic tumors, other primary CNS tumors and other hematological diseases. Tumor resection guided by neuronavigation combining with chemotherapy and radiotherapy may achieve a good effect.

Key words: Leukemia, lymphocytic, chronic, B-cell, Orbit, Neoplasm invasiveness, Immunohistochemistry, Pathology