原发性中枢神经系统NK/T细胞淋巴瘤临床病理分析

Clinical pathological analysis of primary central nervous system extranodal natural killer/T-cell lymphoma

目的探讨原发性CNS NK/T细胞淋巴瘤(PCNSL-NK/T)的临床及病理学特点。方法对本院1例PCNSL-NK/T患者的临床资料及文献报道的12例PCNSL-NK/T患者的资料进行回顾性分析。结果本研究13例PCNSL-NK/T患者的中位发病年龄为39岁,男性多于女性(3.3∶1),亚裔人群多发(9例)。患者临床和影像学表现缺乏特异性,确诊需依赖病理形态学、免疫组化及分子病理检测。本病呈高度侵袭性,缺乏有效治疗,预后极差。此外,本院1例PCNSL-NK/T可表达CD20免疫表型。结论 PCNSL-NK/T是一种十分罕见的高度侵袭性肿瘤,免疫表型也可表达CD20。

Objective To explore the clinic-pathological features of primary central nervous system extranodal natural killer/T-cell lymphoma （PCNSL-NK/T）. Methods The clinical data of one PCNSL-NK/T patient who treated in our hospital and other 12 PCNSL-NK/T patients from literatures were analyzed retrospectively. Results The median onset age of 13 PCNSL-NK/T patients in this study was 39 years, and the male to female ratio was 3.3： 1. In this group, mainly were Asian patients （9 cases）. Clinical and imaging findings were lack of specificity. The accurate diagnosis needed rely on the histomorphology, immunophenotype and molecular technique. The disease was highly aggressive, lack of effective treatment and with poor prognosis. In addition, one case of PCNSL-NK/T patient who treated in our hospital could expressed as CD20. Conclusion The PCNSL-NK/T is extremely rare and highly aggressive, which may express the B cell marker CD20.