Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of primary central nervous system T-cell lymphomas (TPCNSL), and to analyze its biological behavior and prognosis. Methods: Three cases of TPCNSL were collected from September 2014 to September 2019 in the First Affiliated Hospital of Nanjing Medical University. They were evaluated by HE, immunohistochemistry (IHC) and molecular genetics, and the relevant literature was reviewed. Results: Among the 6 816 brain tumors, 97 were primary central nervous system lymphomas (PCNSL), including 3 TPCNSL. There were two male and one female patients, aged 60, 67, and 82 years. Clinically, they were presented with varying degrees of limb numbness and unstable gait. Microscopically, the tumor cells were distributed diffusely or around blood vessels. They showed significant atypia and brisk mitotic activity. By IHC, they were positive for LCA, CD3, CD43, TIA-1, and perforin. Two of three cases were positive for CD5 and granzyme B. T-cell receptor gene rearrangement was clonal. EBER in situ hybridization was negative. The patients were followed for 1 to 6 months; one patient received chemotherapy and died of recurrence 3 months after surgery. One patient died of recurrence 5 months after operation alone. One patient remained recurrence and metastasis free more than 4 months post surgery. Conclusions: PCNSL is uncommon, and most are B-cell lymphomas, while T-cell lymphomas are even rarer. As the latter may show atypical clinical manifestations, diverse histologic morphology and poor prognosis, early diagnosis and timely treatment are particularly important for patients to improve survival.
目的： 探讨原发性中枢神经系统T细胞淋巴瘤（primary central nervous system T-cell lymphomas，TPCNSL）临床病理特征、诊断及鉴别诊断，并分析其生物学行为及预后。 方法： 收集2014年9月至2019年9月南京医科大学第一附属医院收治的经病理确诊的TPCNSL 3例，观察其临床及影像学表现，分析其组织学、免疫组织化学、分子遗传学特点，收集随访结果，并复习相关文献。 结果： 经电子病历系统查询，6 816例脑肿瘤患者中原发性中枢神经系统淋巴瘤97例。其中3例为TPCNSL，男性2例，女性1例，年龄60、67、82岁。临床表现多样，均出现不同程度的肢体乏力、麻木，行走不稳；其中1例伴有烦躁不安、易激惹；1例伴有精神萎靡、嗜睡。镜下肿瘤细胞弥漫分布或围血管生长；瘤细胞异型性明显，可见活跃的核分裂。免疫组织化学3例均阳性表达白细胞共同抗原、CD3、CD43、T细胞胞质内抗原1、穿孔素，2/3例表达CD5、颗粒酶B，Ki-67阳性指数70%~80%。EB病毒编码的小RNA原位杂交均阴性；T细胞受体基因重排均阳性。随访1~6个月，术后仅1例接受化疗，并于术后3个月复发死亡；1例于单纯手术后5个月复发死亡；余1例术后4个月余，未见明确复发及转移征象，后期将继续跟进随访。 结论： TPCNSL非常少见。因临床表现不典型、组织学形态多样，且预后极差，早期明确诊断、及时治疗对于提高患者生存率显得尤为重要。.
Keywords: Central nervous system neoplasms; Diagnosis, differential; Lymphoma, T-cell.