ABSTRACT
Primary extranodal lymphomas are a group of very diverse but very interesting presentations of malignant lymphoma. The natural history of a disease and its management are related to the histological type and to the presenting site. The approach to the management of primary extranodal lymphomas is based on the principles that govern the approach to nodal lymphomas. The management of localized marginal zone primary extranodal lymphomas is usually with involved field radiation therapy. Primary extranodal lymphomas present the clinician with an opportunity to provide curative treatment to the majority of patients. Radiation, given with the guidance of modern imaging techniques and careful dosimetry, is a cornerstone of modern treatment of extranodal lymphomas. The outcomes in localized primary extranodal lymphoma mirror those in nodal lymphomas. For example, the outcomes in primary orbital lymphomas are excellent, primarily because marginal zone lymphoma represents the majority of cases.
