Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a lymphoid malignancy that mainly affects the nasopharynx and is associated with the Epstein-Barr virus (EBV). Increased incidence is seen in some Latin American and Asian countries. In this study, we describe a case series of 86 Guatemalan patients with ENKTCL-NT from a single diagnostic head and neck center. We emphasize the distinctive clinical, microscopic, and immunohistochemical (IHC) features, as well as EBV positivity by in situ hybridization (ISH). Most of the patients (90.6%) were of Mayan descent and low socioeconomic status (SES). Males were more often affected than females, comprising 68.3% of cases. Patient age ranged from 8 to 71, with a mean of 34.7 years. All cases arose in the upper aerodigestive tract and mainly presented as a rapidly progressive, necrotizing midfacial process affecting the nasal, nasopharyngeal, sinonasal, palatal, and oropharyngeal structures. Microscopically, ENKTCL-NT showed a diffuse polymorphic and atypical lymphoid infiltrate. Angiocentric and angiodestructive growth patterns were present with associated necrosis. Peripheral hyaline necrosis of blood vessels was a histologic hallmark. The ISH and IHC profiles included positivity of EBV, LCA, CD3, CD45RO, CD30 (focal in 39.2%), granzyme-B, TIA-1, perforin (in 82.3%), and CD56 (in 83.7%). CD20 was negative, and the Ki-67 index ranged from 70 to 90%. In Guatemala, this lymphoma is strongly associated with people of low SES and indigenous ethnicity. When affected, the palatal mucosa provides the best site to obtain a representative biopsy. Since ENKTCL-NT is highly aggressive, it is extremely important to recognize the spectrum of clinical presentations and microscopic features in order to avoid misdiagnosis and treatment delay.
