Successful Treatment of Primary CNS Extranodal NK/T-Cell Lymphoma with Surgery and Chemotherapy Combined with Sintilimab: A Case Report and Literature Review

Liping Qin,1,2,* Yajun Li,2,* Yizi He,2 Ruolan Zeng,2 Tao Pan,2 Yilang Zuo,2 Ling Xiao,3 Hui Zhou2 1Graduate Collaborative Training Base of Hunan Cancer Hospital, Hengyang Medical School, University of South China, Hengyang, Hunan, 421001, People’s Republic of China; 2Department 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; 3Department of Histology and Embryology of School of Basic Medical Science, Central South University, Changsha, Hunan, 410013, People’s Republic of China*These authors contributed equally to this workCorrespondence: Hui ZhouDepartment 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 ChinaTel +86-73189762281Email zhouhui9403@126.comLing XiaoDepartment of Histology and Embryology of School of Basic Medical Science, Central South University, Changsha, Hunan, 410013, People’s Republic of ChinaTel +86-73182650436Email xiaolingcsu@csu.edu.cnAbstract: 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.Keywords: primary central nervous system, extranodal natural killer/T-cell lymphoma, chemotherapy, immunotherapy, PD1