ABSTRACT
INTRODUCTION: Primary nasal T/NK cell lymphoma is a very rare pathological clinical entity; it was defined by the WHO in 2001, thanks to immunohistochemistry. The treatment combines radiotherapy and chemotherapy. We report a case. CASE REPORT: A 20-year-old male patient was admitted for ulceration of the vestibular mucosa from tooth 20 to 26, in June 2008. The lesion had appeared four months before, with a painful ulceration close to tooth 25. The lesion evolved progressively, it eroded the alveolar bone, exposing the roots of teeth 24, 25, and 26. CT scan revealed lysis of the external maxillary sinus wall. The diagnosis of T/NK cell lymphoma was obtained by immunohistochemistry of the biopsy. The tumor was staged IeA according to the Ann Arbor classification. After four courses of CHOP chemotherapy and two years of follow-up there was no recurrence. DISCUSSION: Maxillary T/NK cell lymphoma is extremely rare. The diagnosis is based on immunohistochemistry. The treatment associates chemotherapy and radiotherapy. Recent studies suggest that radiotherapy at an early stage could improve the prognosis. But there is no consensus on therapeutic protocols.