Extranodal NK/T-cell lymphoma, nasal type: report of 15 cases

To define the epidemiological and clinical features and complementary investigation findings of extranodal NK/T-cell lymphoma, nasal type and to discuss the diagnostic difficulties and the various treatment options.

This retrospective study was based on 15 patients with extranodal NK/T-cell lymphoma, nasal type, managed between 1990 and 2009.

This series comprised 13 men and two women (sex ratio=6.5) with a mean age of 52 years (range: 35-81 years). The mean time to first consultation was 6 months. The most common symptoms were nasal obstruction (87%) and purulent nasal discharge (73%), followed by epistaxis (60%). Physical examination demonstrated the presence of a tumour of the nasal cavity in 11 patients. The diagnosis was confirmed by histological examination of a biopsy completed by immunohistochemistry. CT scan of the facial bones was performed in all patients of this series. The site of extranodal NK/T-cell lymphoma was essentially nasal (12 cases). Orbital extension was observed in four cases, associated with intracranial extension in two cases and osteolysis was observed in 11 patients. Lymphomas were classified as stage IE in 74% of cases and stage IIE in 26% of cases. Only one patient was lost to follow-up during treatment. Three patients died before any treatment. Treatment therefore concerned 12 patients. Stage IE lymphomas were treated by radiotherapy and/or chemotherapy. All stage IIE lymphomas were treated by chemotherapy alone. Stage IE patients had a better prognosis.

Extranodal NK/T-cell lymphoma, nasal type, is an aggressive form of non-Hodgkin's lymphoma comprising specific clinicopathological characteristics. The addition of chemotherapy for advanced stages does not appear to improve survival compared radiotherapy alone, which remains the treatment of choice especially for localized stages.