Immunophenotypic and clinical differences between the nasal and extranasal subtypes of upper aerodigestive tract natural killer/T-cell lymphoma.
OBJECTIVE
To investigate, in a large cohort of patients, the immunophenotypic and clinical differences of nasal and extranasal extranodal nasal-type natural killer/T-cell lymphoma of the upper aerodigestive tract (UADT-NKTCL) and examine the relevance of the immunophenotype on the clinical behavior, prognosis, and treatment.


METHODS
A total of 231 patients with UADT-NKTCL were recruited. One hundred eighty-one patients had primary location in the nasal cavity (nasal UADT-NKTCL), and 50 patients had primary extranasal UADT-NKTCL.


RESULTS
Patients with extranasal UADT-NKTCL had more adverse clinical features, including advanced-stage disease, regional lymph node involvement, B symptoms, and poor performance status, than patients with nasal UADT-NKTCL. In addition, CD56 and granzyme B were less frequently expressed in extranasal UADT-NKTCL. The 5-year overall survival rate was 74.1% for the entire group and 76.0% for early-stage disease. The 5-year overall survival rate for extranasal UADT-NKTCL was similar or superior to that of nasal UADT-NKTCL for all disease stages (76.9% vs 73.4%, P=.465), stage I disease (75.9% vs 79.2%, P=.786), and stage II disease (83.3% vs 50.3%, P=.018). CD56 expression and a Ki-67 proliferation rate ≥ 50% predicted poorer survival for extranasal UADT-NKTCL but not for nasal UADT-NKTCL.


CONCLUSIONS
Patients with nasal and extranasal UADT-NKTCL have significantly different clinical features, immunophenotypes, and prognosis. Extranasal UADT-NKTCL should be considered as a distinct subgroup apart from the most commonly diagnosed prototype of nasal UADT-NKTCL.
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Qing-Feng
QF
Liu
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Wei-Hu
WH
Wang
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Shu-Lian
SL
Wang
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Yue-Ping
YP
Liu
Department of Pathology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Wen-Ting
WT
Huang
Department of Pathology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Ning
N
Lu
Department of Medical Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Li-Qiang
LQ
Zhou
Department of Radiology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Han
H
Ouyang
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.
Jing
J
Jin
Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China. Electronic address: yexiong@yahoo.com.
Ye-Xiong
YX
Li
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000951:Antigens, Neoplasm / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D019002:CD56 Antigen / Q000378:metabolism; D002648:Child; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016130:Immunophenotyping / Q000379:methods*; D019394:Ki-67 Antigen / Q000378:metabolism; D007822:Laryngeal Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D008207:Lymphatic Metastasis; D054391:Lymphoma, Extranodal NK-T-Cell / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D010610:Pharyngeal Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D014062:Tongue Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D014134:Tracheal Neoplasms / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
D000951:Antigens, Neoplasm; D019002:CD56 Antigen; D019394:Ki-67 Antigen; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone