A case of primary cutaneous nasal type NK/T-cell lymphoma and review of the literature.

ABSTRACT

INTRODUCTION: Cutaneous T-cell lymphoma subtypes are now better identified thanks to progress in immunohistochemistry. This article describes a new case of primary cutaneous natural killer/T-cell lymphoma of nasal type (NKTL-NT) and reviews 18 other cases of this rare neoplasm. CASE REPORT A 79-year-old man presented with a 3-cm nodular tumor of the left leg occurring on a primary chronic lymphedema of the legs. The lesion was CD56+, CD3 intracytoplasmic+, CD45+ and Epstein-Barr virus+. A comprehensive workup including CT scan and bone marrow biopsy was negative and a diagnosis of NKTL-NT with a primary cutaneous involvement was made. The patient was free of disease under multi-agent chemotherapy after 24 months of follow-up. DISCUSSION: After reviewing 18 other cases of primary cutaneous NKTL-NT, we conclude that the prognosis of these lymphomas is usually poor. However, limited cutaneous forms have a longer median survival than extracutaneous variants.