医学
恶性肿瘤
淋巴瘤
放射治疗
T细胞淋巴瘤
病因学
病理
皮肤病科
内科学
作者
Celeste Sánchez‐Romero,Ronell Bologna‐Molina,Oslei Paes de Almeida,Alan Roger Santos‐Silva,Ana Carolina Prado‐Ribeiro,Thaís Bianca Brandão,Román Carlos
标识
DOI:10.1016/j.critrevonc.2021.103237
摘要
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is an aggressive malignancy associated with Epstein-Barr virus infection, with a geographic and racial predilection for some Asian and Latin American countries. ENKTCL-NT manifests as a necrotic process affecting nasal or upper aerodigestive structures and, rarely, extranasal sites such as skin, and the gastrointestinal tract. ENKTCL-NT was characterized by its poor prognosis irrespective of clinical stage and therapy. However, during the last two decades, advances in its clinicopathologic, genetic and molecular characterization have been achieved, as have changes in the chemotherapy regimens that, in combination with radiotherapy, are significantly improving the survival of these patients, especially in initial stages. For these reasons, we present an overview of the historical background of ENKTCL-NT along with an updated review of its potential etiological factors, clinicopathologic and molecular features, as well as its prognostic models, current treatment protocols, and future directions on potential promising therapeutic approaches.
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