Introduction: Extra-nodal T lymphomas of the ear, nose, and throat (ENT) are unusual in Western countries, with differential diagnosis from other destructive and necrotizing lesions of the sino-nasal tract often difficult.
Material and methods: Eleven cases of extra-nodal lymphomas of the upper aerodigestive tract tract managed in the ENT department of F. Hached hospital in Sousse, Tunisia, were retrieved over a 10-year period (1995-2004). The aim of our study was to report the clinical and pathological data and the outcome for each patient.
Results: The median age of patients was 41-years. The most frequent site of involvement was the nasal cavity. Clinically, the symptoms were not specific. Histological examination identified two cases of T/NK lymphoma and nine cases of T-lymphoma not otherwise specified. Immunohistochemistry and in situ hybridization techniques positively detected Epstein-Barr virus in tumoral cells, in seven cases. Tumor staging, in 10 patients, found distant metastasis in three. Nine patients underwent the following treatment: exclusive radiotherapy (one patient), radiochemotherapy (three patients), and exclusive polychemotherapy (five patients). The two remaining patients had no treatment because of a rapidly unfavorable progression. After a median follow-up of 11-months, clinical outcome consisted in death in seven patients and remission in three patients; one patient was lost of follow-up.
Conclusion: Extra-nodal lymphomas of the ORL tract are rare in Tunisia. Clinical presentation is not specific, depending on the lymphoma location. Diagnosis relies on clinical presentation and immunophenotypic and molecular characteristics; morphological features are not specific. These are aggressive lymphomas, often requiring multidisciplinary management.