Non-Hodgkin's lymphoma of the sino-nasal tract in children.

Childhood head and neck cancers are relatively uncommon. Of all head and neck cancers occurring in children, non-Hodgkin's lymphoma (NHL) is the most common, others being rhabdomyosarcoma and nasopharyngeal carcinoma. In the head and neck region, sinuses are the second commonest primary site of NHL after neck lymph nodes. These can be of several different types depending on the predominant cell type and histologic appearance, the most common histological variant being diffuse large B-cell lymphoma. In an attempt to simplify the classification and to develop a universally acceptable classification and staging, they have been classified and staged numerous times over the last three decades, adding more confusion to the topic. Clinical presentations vary according to the histological type. The low grade lymphomas present with a nasal cavity or para-nasal sinus mass associated with obstructive symptoms and/or lymphadenopathy, while high grade lymphomas present with aggressive signs and symptoms including non-healing ulcer, epistaxis, septal perforation and bony destruction. The primary treatment consists of chemotherapy and / or radiation therapy, which is able to achieve remission in two-third of the patients, however, prognosis remains poor with cumulative five-year survival rates at about 30% for all the types of sino-nasal NHLs. Newer targeted therapy (monoclonal antibodies) and combination therapies (including stem cells) are currently being tested in order to improve survival rates in these patients. This article aims at providing an overview of clinico-epidemiologic characteristics, staging system currently in use, management, prognosis and possibilities of future research in the field of childhood sinonasal NHLs.