Qin Liping, Li Yajun, He Yizi, Zeng Ruolan, Pan Tao, Zuo Yilang, Xiao Ling, Zhou Hui
Graduate Collaborative Training Base of Hunan Cancer Hospital, Hengyang Medical School, University of South China, Hengyang, Hunan, 421001, People's Republic of China.
Department of Lymphoma and Hematology, Hunan Cancer Hospital and The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, Hunan, 410013, People's Republic of China.
Onco Targets Ther. 2022 Jan 6;15:1-11. doi: 10.2147/OTT.S343400. eCollection 2022.
Primary central nervous system extranodal natural killer/T-cell lymphoma (PCNS ENK/TCL) is an extremely rare lymphoma. Only 23 cases of PCNS ENK/TCL have been reported in the English literature. Due to the rarity of this lymphoma, an effective therapeutic strategy has not been defined. Generally, this type of lymphoma is treated with surgery, intrathecal chemotherapy, and postoperative chemoradiation therapy. The prognosis is poor. Herein, we present a case of primary brain NK/T cell lymphoma in a 50-year-old immunocompetent Chinese female and review the literature. The patient underwent intracranial tumor resection and was subsequently treated with a PD1 monoclonal antibody (Sintilimab) combined with chemotherapy. The patient survived 15 months after diagnosis. This is the first report of PCNS ENK/TCL treated with surgery and chemotherapy combined with immunotherapy and suggests an effective treatment regimen for PCNS ENK/TCL.
原发性中枢神经系统结外自然杀伤细胞/ T细胞淋巴瘤（PCNS ENK/TCL）是一种极其罕见的淋巴瘤。英文文献中仅报道了23例PCNS ENK/TCL。由于这种淋巴瘤罕见，尚未确定有效的治疗策略。一般来说，这类淋巴瘤采用手术、鞘内化疗及术后放化疗进行治疗。其预后较差。在此，我们报告一例50岁免疫功能正常的中国女性原发性脑NK/T细胞淋巴瘤病例并复习相关文献。该患者接受了颅内肿瘤切除术，随后接受了PD1单克隆抗体（信迪利单抗）联合化疗。患者确诊后存活了15个月。这是首例关于PCNS ENK/TCL采用手术、化疗联合免疫治疗的报道，提示了一种针对PCNS ENK/TCL的有效治疗方案。
