Extranodal malignant lymphomas of the head and neck

The management of primary extranodal lymphomas of the head and neck at The Princess Margaret Hospital is reviewed. This manner of presentation is uncommon and particularly rare in Hodgkin's disease. As with lymphomas at any other site, detailed investigation is necessary to determine the stage of the disease before definitive treatment is attempted. Surgery is normally restricted to biopsy. Radiation therapy and chemotherapy, alone or combined are the principal treatment modalities. Early disease (stages IA and IIA) may be treated by radiation alone with a cause specific survival of approximately 70% at five years and 60% at 10 years. Survival is better for histiocytic, nodular, and less bulky disease. Primary thyroid disease is uncommon but highly curable with local radiation. Similarly, orbital disease is usually locally curable with negligible sequelae. The addition of combination chemotherapy has significantly improved the prognosis with more advanced and bulky disease.