Nasal angiocentric T cell-natural killer cell lymphoma with pancreatic localisation in a child

The nasal angiocentric T-cell-natural killer cell lymphoma is an aggressive tumor, exceptional in Tunisia, which is observed especially in Asia, Mexico and South America. We report the case of an 11-year-old boy, presenting with an exuberant and ulcerative tumor of the right nasal pit. Radiological investigation evidenced a right ethmoi¨do-frontal tumor. Evolution was characterized by the installation of abdominal pain and fever, the evidenciation of a mass within the head of the pancreas that compressed the extra hepatic biliary duct and Wirsung’s canal and was associated to hyperamylasemia. Biopsy under ultrasound revealed a histiocytic nature. Nasal biopsy found histiocytic and lymphocytic cells and led to a diagnosis of rhinoscleroma. The absence of improvement under antibiotic treatment and the development of fever with leuconeutropenia oriented towards the diagnosis of a non-Hodgkinian malignant lymphoma. Medullary biopsy and immunohistochimic study confirmed a T/NK cells lymphoma, with medullary invasion. The child died from acute respiratory distress syndrome. The authors insist on the rarity of this bipolar location, in particular in children, emphasize the difficulty of the diagnosis and review the literature. [Copyright &y& Elsevier]