BACKGROUND: This study was conducted to determine whether the paranasal extension of a primary non-Hodgkin's lymphoma (NHL) of the nasal cavity has any deleterious effect on patient outcome. METHODS: One hundred and seventy-five patients with previously untreated nasal NHL were reviewed. There were 2 with low grade, 107 with intermediate grade, 17 with high grade, and 49 with unclassifiable lymphomas. In 48 cases the immunophenotype was available and 46 were T-cell lymphoma. According to the Ann Arbor system, there were 133 patients with Stage IE, 28 with Stage IIE, 4 with Stage IIIE, and 10 with Stage IVE lymphomas. Stage IE was subdivided into limited Stage IE (i.e., confined to the nasal cavity [67 patients]) or extensive Stage IE (i.e., presenting with extension beyond the nasal cavity [66 patients]). For patients with limited Stage IE disease the treatment of choice was radiotherapy with or without chemotherapy. In patients with extensive Stage IE disease, treatment was comprised of a combination of chemotherapy and radiotherapy or radiotherapy alone. For patients with a more advanced stage of disease (IIE-IVE), chemotherapy was an integral part of the treatment and was completed by irradiation, especially for patients with Stage IIE disease. RESULTS: The actuarial overall survival (OS) and disease free survival (DFS) rates at 5 years for the whole group were 65% and 57%, respectively. The 5-year OS and DFS rates were influenced by stage, with a gradual decrease from 75% and 68% for Stage IE disease to 35% and 28% for Stage IIE disease, and 31% and 19% for Stage IIIE/IVE disease. Patients with limited Stage IE disease survived significantly longer (90% 5-year OS) compared with those with extensive Stage IE disease (57% 5-year OS; P < 0.001). For 67 patients with limited Stage IE disease, the 5-year OS was 89% with radiotherapy alone and 92% with radiotherapy and chemotherapy, whereas for 66 patients with extensive Stage IE disease, the 5-year OS was 54% with radiotherapy and 58% with combined modality therapy or chemotherapy (P > 0.05). CONCLUSIONS: The prognosis of patients with primary NHL of the nasal cavity is stage dependent. In this large cohort of Stage IE patients, it was demonstrated that the paranasal local extension was a significant prognostic factor associated with poorer treatment outcome. The authors believe that Ann Arbor Stage IE should be subclassified further into limited and extensive Stage IE. The addition of chemotherapy did not appear to modify significantly the survival of patients with either limited or extensive Stage IE disease. The extranodal progression observed in patients with extensive Stage IE and Stage IIE-IVE disease clearly illustrates the need for improvement of systemic treatment.