Extranodal natural killer/T cell lymphomas with extranasal disease in non-endemic regions are disseminated or have nasal primary: a study of 84 cases from India.

Abstract We report a study of 84 extranodal natural killer/T cell lymphomas (ENKTCLs) from India to understand the disease pattern. Sixty-one patients had nasal disease and three had Waldeyer's ring tumors, while 20 had extranasal sites of presentation, namely: cervix (n = 1), oral cavity (n = 3), orbit (n = 3), liver (n = 1), ileum (n = 1), and soft tissue and lymph nodes (n = 9). Staging of patients with extranasal disease revealed that either they had disseminated disease or the nasal mass was missed during the initial staging. Extranasal presentation, performance status (PS), international prognostic index (IPI), Korean prognostic index, marrow involvement, radiotherapy and type of chemotherapy affected overall survival, while lymphopenia, performance status, radiotherapy and type of chemotherapy impacted disease-free survival. In the multivariate analysis, IPI and response to chemotherapy emerged as significant prognostic factors. Thus most patients with extranasal ENKTCL have a nasal lesion or disseminated disease and pure extranasal disease is uncommon in non-endemic regions.