Abstract

Background: Extranodal nasal-type NK/T-cell lymphoma (ENKTCL) is a common and geographic predominant disease in China. We observed the clinical and pathologic features of this tumor type and its relationship to Epstein-Barr virus (EBV), and focused on the epidermology and prevalence of ENKTCL in Xinjiang, in order to improve the diagnostic and differential diagnosis ability.
Methods: 103 cases with nasal-type NK/T-cell lymphoma were recruited and assessed. All cases were submitted for histological assessment, immunophenotyping, in situ hybridization detection of EBV-encoded RNA (EBER), and clinical information analysis.
Results: Male patients, aged between 40 and 50 years, were predominantly of Han ethnicity outnumbering those of Uyghur ethnicity. 80 cases of ENKTCL involved the nasal cavity or other areas of the upper respirodigestive tract whereas the remaining ones involved other extranodal sites. All cases exhibited similar histological changes: necrosis and inflammatory infiltration were frequently present. Cytologic atypical as well as angiocentric and angiodestructive growth patterns of the tumor cells were consistently noted, although the sizes and contours of the tumor cells varied considerably. All cases expressed one or several T-cell markers and at least one cytotoxic molecule. 86 cases (84%) expressed CD56, and 97 cases submitted for EBER detection showed positive results. ENKTCL affects predominantly middle-aged Han Chinese in the Xinjiang region, with a predilection to involvement of the nasal cavity and a strong association with a high EBV load.
Conclusions: Early and correct identification of the disease in daily practice depends on the combination of morphologic evaluation, immunophenotypic assessment, and EBER detection.

DOI: 10.7754/Clin.Lab.2013.130236