title: Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents.
abstract: Extranodal nasal-type natural killer (NK)/T-cell lymphoma is rarely observed in children and adolescents. We aim to investigate the clinical features, prognosis, and treatment outcomes in these patients. Thirty-seven patients were reviewed. There were 19, 14, 2, and 2 patients with stage I, stage II, stage III, and stage IV diseases, respectively. Among the patients with stage I and II disease, 19 patients received initial radiotherapy with or without chemotherapy, and 14 patients received chemotherapy followed by radiotherapy. The 4 patients with stage III and IV disease received primary chemotherapy and radiation of the primary tumor. Children and adolescents with extranodal nasal-type NK/T-cell lymphoma usually presented with early-stage disease, high frequency of B symptoms, good performance, low-risk age-adjusted international prognostic index, and chemoresistance. The complete response rate after initial radiotherapy was 73.7%, which was significantly higher than the response rate after initial chemotherapy (16.7%; P = .002). The 5-year overall survival (OS) and progression-free survival (PFS) rates for all the patients were 77.0% and 68.5%, respectively. The corresponding OS and PFS rates for patients with stage I and II disease were 77.6% and 72.3%, respectively. Children and adolescents with early-stage extranodal nasal-type NK/T-cell lymphoma treated with primary radiotherapy had a favorable prognosis.
authors.affiliation: Department of Radiation Oncology, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
authors.forename: Zhao-Yang
authors.initials: ZY
authors.lastname: Wang
authors.forename: Ye-Xiong
authors.initials: YX
authors.lastname: Li
authors.forename: Wei-Hu
authors.initials: WH
authors.lastname: Wang
authors.forename: Jing
authors.initials: J
authors.lastname: Jin
authors.forename: Hua
authors.initials: H
authors.lastname: Wang
authors.forename: Yong-Wen
authors.initials: YW
authors.lastname: Song
authors.forename: Qing-Feng
authors.initials: QF
authors.lastname: Liu
authors.forename: Shu-Lian
authors.initials: SL
authors.lastname: Wang
authors.forename: Yue-Ping
authors.initials: YP
authors.lastname: Liu
authors.forename: Shu-Nan
authors.initials: SN
authors.lastname: Qi
authors.forename: Hui
authors.initials: H
authors.lastname: Fang
authors.forename: Xin-Fan
authors.initials: XF
authors.lastname: Liu
authors.forename: Zi-Hao
authors.initials: ZH
authors.lastname: Yu
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002681:China; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D054391:Lymphoma, Extranodal NK-T-Cell / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy*; D008297:Male; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy*; D010610:Pharyngeal Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy*; D011241:Prednisone / Q000008:administration & dosage; D050397:Radiotherapy, Intensity-Modulated; D012074:Remission Induction; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone