Extranodal NK/T cell lymphoma nasal type (ENKTL) is a rare malignancy. Due to its non-specific early symptoms and highly aggressive nature, the diagnosis and treatment of ENKTL remains clinically challenging. Combination of chemotherapy and radiotherapy is the standard treatment for stage Ⅰ/Ⅱ ENKTL. Herein, we report a case of primary ENKTL first presented in left-side nasal cavity and then in right-side larynx. More than four years after the initial diagnosis of nasal ENKTL, his left nose was ulcerated due to swelling and it was highly suspected that the lesion was local recurrence. Following up for 22 months after the diagnosis of laryngeal ENKTL, there was no sign of local recurrence of laryngeal lesions after six cycles of GDP program and three-dimensional radiotherapy. The final diagnosis was primary laryngeal ENKTL, and local recurrence of nasal cavity was highly suspected. From the initial diagnosis, after the application of CHOPE, DDGP/DGP sequential chemotherapy and simultaneous radiotherapy, nasal and laryngeal tumors were successfully controlled. We also review the relevant literature to discuss the treatment of NKT, hoping to provide some clinical enlightenment.

Chuyun Xing, Shizhou Cheng, Qing Xu, Yanhua Xu, Qin Qin. Extranodal NK/T cell lymphoma nasal type: A case report and literature review. Frontiers in Medical Science Research (2022) Vol. 4, Issue 13: 52-56. https://doi.org/10.25236/FMSR.2022.041309.

[1] Mundy-Bosse B, Weinstock DM. Breaking Down the Barriers to Define and Treat NK/T Cell Lymphoma. Cancer Cell 2020, 37(3):263-265.

[2] Tse E, Kwong YL. The diagnosis and management of NK/T-cell lymphomas. J Hematol Oncol 2017, 10(1):85.

[3] de Mel S, Hue SS, Jeyasekharan AD, Chng WJ, Ng SB. Molecular pathogenic pathways in extranodal NK/T cell lymphoma. J Hematol Oncol 2019, 12(1):33.

[4] Lee J, Au WY, Park MJ, Suzumiya J, Nakamura S, Kameoka J, Sakai C, Oshimi K, Kwong YL, Liang R et al. Autologous hematopoietic stem cell transplantation in extranodal natural killer/T cell lymphoma: a multinational, multicenter, matched controlled study. Biol Blood Marrow Transplant 2008, 14(12):1356-1364.

[5] Kwong YL, Chan TSY, Tan D, Kim SJ, Poon LM, Mow B, Khong PL, Loong F, Au-Yeung R, Iqbal J et al. PD1 blockade with pembrolizumab is highly effective in relapsed or refractory NK/T-cell lymphoma failing l-asparaginase. Blood 2017, 129(17):2437-2442.

[6] Wang L, Li LR, Zhang L, Wang JW. The landscape of new drugs in extranodal NK/T-cell lymphoma. Cancer Treat Rev 2020, 89:102065.

[7] Pokrovsky VS, Vinnikov D. Defining the toxicity of current regimens for extranodal NK/T cell lymphoma: a systematic review and metaproportion. Expert Rev Anticancer Ther 2019, 19(1):93-104.

[8] Yamaguchi M, Suzuki R, Oguchi M: Advances in the treatment of extranodal NK/T-cell lymphoma, nasal type. Blood 2018, 131(23):2528-2540.

[9] Huang H, Lin Z, Lin X, Cai Q, Xia Z, Jiang W. Long-term outcomes of patients with newly diagnosed extranodal natural killer/T-cell lymphoma treated by etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin regimen: a single-institution experience. Leuk Lymphoma 2011, 52(6):1041-1048.

[10] Moon SH, Cho SK, Kim WS, Kim SJ, Chan Ahn Y, Choe YS, Lee KH, Kim BT, Choi JY. The role of 18F-FDG PET/CT for initial staging of nasal type natural killer/T-cell lymphoma: a comparison with conventional staging methods. J Nucl Med 2013, 54(7):1039-1044.

[11] Zhou X, Lu K, Geng L, Li X, Jiang Y, Wang X. Utility of PET/CT in the diagnosis and staging of extranodal natural killer/T-cell lymphoma: a systematic review and meta-analysis. Medicine (Baltimore) 2014, 93(28):e258.

[12] Gungor A, Pennington L, Sankararaman S, Zaid-Kaylani S, Jeroudi MA. A 14-year-old boy with extranodal natural killer cell lymphoma of the nose, nasopharynx, larynx, and trachea in remission 6years after primary diagnosis. A longitudinal case report. Am J Otolaryngol 2016, 37(6):563-566.

[13] Tse E, Kwong YL. How I treat NK/T-cell lymphomas. Blood 2013, 121(25):4997-5005.

[14] Lin N, Song Y, Zheng W, Tu M, Xie Y, Wang X, Ping L, Ying Z, Zhang C, Deng L et al. A prospective phase II study of L-asparaginase- CHOP plus radiation in newly diagnosed extranodal NK/T-cell lymphoma, nasal type. J Hematol Oncol 2013, 6:44.

[15] Li X, Cui Y, Sun Z, Zhang L, Li L, Wang X, Wu J, Fu X, Ma W, Zhang X et al. DDGP versus SMILE in Newly Diagnosed Advanced Natural Killer/T-Cell Lymphoma: A Randomized Controlled, Multicenter, Open-label Study in China. Clin Cancer Res 2016, 22(21):5223-5228.

[16] Liu T, Zhu F, Xiao Y, Li Q, Liu X, Yang K, Wu G, Zhang L. Pegaspargase, gemcitabine, dexamethasone, and cisplatin (P-GDP) combined chemotherapy is effective for newly diagnosed extranodal NK/T-cell lymphoma: a retrospective study. Cancer Manag Res 2018, 10:5061-5069.

[17] Tian S, Li R, Wang T, Wang S, Tao R, Hu X, Ding H. Gemcitabine, dexamethasone, and cisplatin (GDP) chemotherapy with sandwiched radiotherapy in the treatment of newly diagnosed stage IE/IIE extranodal natural killer/T-cell lymphoma, nasal type. Cancer Med 2019, 8(7):3349-3358.

[18] Li YX, Liu QF, Wang WH, Jin J, Song YW, Wang SL, Liu YP, Liu XF, Zhou LQ, Yu ZH. Failure patterns and clinical implications in early stage nasal natural killer/T-cell lymphoma treated with primary radiotherapy. Cancer 2011, 117(22):5203-5211.

[19] Wang H, Wuxiao ZJ, Zhu J, Wang Z, Wang KF, Li S, Chen X, Lu Y, Xia ZJ. Comparison of gemcitabine, oxaliplatin and L-asparaginase and etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone as first-line chemotherapy in patients with stage IE to IIE extranodal natural killer/T-cell lymphoma: a multicenter retrospective study. Leuk Lymphoma 2015, 56(4):971-977.

[20] Wang JH, Wang L, Liu CC, Xia ZJ, Huang HQ, Lin TY, Jiang WQ, Lu Y. Efficacy of combined gemcitabine, oxaliplatin and pegaspargase (P-gemox regimen) in patients with newly diagnosed advanced-stage or relapsed/refractory extranodal NK/T-cell lymphoma. Oncotarget 2016, 7(20): 29092-29101.