TY - JOUR
T1 - Extranodal NK/T-cell lymphoma, nasal type
T2 - A report of 73 cases at MD anderson cancer center
AU - Li, Shaoying
AU - Feng, Xiaoli
AU - Li, Ting
AU - Zhang, Shuang
AU - Zuo, Zhuang
AU - Lin, Pei
AU - Konoplev, Sergej Naumovich
AU - Bueso-Ramos, Carlos E
AU - Vega, Francisco
AU - Medeiros, L Jeffrey
AU - Yin, Cheng Cameron
PY - 2013/1
Y1 - 2013/1
N2 - Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is uncommon in the United States. We report 73 patients with ENKTL, including 49 men and 24 women (median age, 46 y). Sixty-three patients had nasal/upper aerodigestive tract disease; 10 had extranasal disease involving skin, small intestine, epiglottis, testis, adrenal glands, kidney, and breast. Complete staging data were available for 68 patients: 44 stage I/II and 24 stage IV. Fifteen of 69 (22%) had lymphadenopathy and 10/63 had bone marrow involvement. Histologically, 67/73 (92%) showed necrosis, and 48/70 (69%) had an angiocentric/angiodestructive growth pattern. The neoplastic cells showed a wide spectrum: medium sized (n=34), mixed small and large (n=21), large (n=13), and small (n=5). In situ hybridization for Epstein-Barr virus-encoded small RNA was positive in every case. Immunohistochemical studies showed expression of cytotoxic markers (100%), T-bet (96%), CD2 (96%), CD3 (93%), CD56 (90%), and ETS-1 (64%). Ki-67 was ≥60% in 46% cases. Therapy was known for 64 patients; 14 received only chemotherapy, 8 radiation alone, and 42 received combined radiation and chemotherapy. Median survival was 4.2 years, and 5-year overall survival was 46% (median follow-up, 3.8 y). Extranasal disease, high International Prognostic Index score, and high proliferation rate correlated with poorer prognosis. We conclude that ENKTL cases in the United States are similar to those reported in Asia and other countries. Absence of the angiocentric/angiodestructive pattern and presence of lymphadenopathy, features underemphasized in the literature, occurred in appreciable subsets of patients. The International Prognostic Index score, anatomic site of disease, and proliferation rate had prognostic value in this patient cohort.
AB - Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is uncommon in the United States. We report 73 patients with ENKTL, including 49 men and 24 women (median age, 46 y). Sixty-three patients had nasal/upper aerodigestive tract disease; 10 had extranasal disease involving skin, small intestine, epiglottis, testis, adrenal glands, kidney, and breast. Complete staging data were available for 68 patients: 44 stage I/II and 24 stage IV. Fifteen of 69 (22%) had lymphadenopathy and 10/63 had bone marrow involvement. Histologically, 67/73 (92%) showed necrosis, and 48/70 (69%) had an angiocentric/angiodestructive growth pattern. The neoplastic cells showed a wide spectrum: medium sized (n=34), mixed small and large (n=21), large (n=13), and small (n=5). In situ hybridization for Epstein-Barr virus-encoded small RNA was positive in every case. Immunohistochemical studies showed expression of cytotoxic markers (100%), T-bet (96%), CD2 (96%), CD3 (93%), CD56 (90%), and ETS-1 (64%). Ki-67 was ≥60% in 46% cases. Therapy was known for 64 patients; 14 received only chemotherapy, 8 radiation alone, and 42 received combined radiation and chemotherapy. Median survival was 4.2 years, and 5-year overall survival was 46% (median follow-up, 3.8 y). Extranasal disease, high International Prognostic Index score, and high proliferation rate correlated with poorer prognosis. We conclude that ENKTL cases in the United States are similar to those reported in Asia and other countries. Absence of the angiocentric/angiodestructive pattern and presence of lymphadenopathy, features underemphasized in the literature, occurred in appreciable subsets of patients. The International Prognostic Index score, anatomic site of disease, and proliferation rate had prognostic value in this patient cohort.
KW - extranodal NK/T-cell lymphoma
KW - nasal type
KW - southern United States
UR - http://www.scopus.com/inward/record.url?scp=84871614389&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84871614389&partnerID=8YFLogxK
U2 - 10.1097/PAS.0b013e31826731b5
DO - 10.1097/PAS.0b013e31826731b5
M3 - Article
C2 - 23232851
AN - SCOPUS:84871614389
SN - 0147-5185
VL - 37
SP - 14
EP - 23
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 1
ER -