NON-HODGKINS-LYMPHOMA OF THE PARANASAL SINUSES - CLINICAL AND PATHOLOGICAL FEATURES, AND RESPONSE TO COMBINED-MODALITY THERAPY

PURPOSE Lymphomas of the paranasal sinuses may have poorer prognoses c ompared with other extranodal lymphomas of the head and neck, and are not well defined as a particular clinicopathologic entity. The outcome of combined-modality therapy and central nervous system (CNS) prophyl axis has not been fully determined. PATIENTS AND METHODS We retrospect ively reviewed our experience with 16 consecutive, carefully defined p atients, all treated with both chemotherapy and radiotherapy. RESULTS There were 11 men and five women, mean age 52. All presented with loca l symptoms; 13 had stage I or II disease. Thirteen had diffuse large c ell lymphoma, two diffuse mixed, and one small noncleaved. Phenotyping revealed 10 B-cell, four T-cell, and two T or natural killer (NK). Mo st received CHOP (cyclophosphamide, doxorubicin, vincristine, predniso ne) chemotherapy; the order of chemotherapy and radiotherapy varied. T welve received CNS prophylaxis. Of 12 complete responses, six relapsed , all at distant sites, and two died during initial therapy. Five-year survival was 29%, and median survival 18 months. Four of 10 B-lineage patients were relapse-free at 4 years; all six T- or T/NK-lineage pat ients relapsed or were dead within 6 months. Tumors of T or NK lineage often expressed CD56 and showed evidence of Epstein-Barr viral infect ion; otherwise, pathological features were not predictive of lineage o r outcome. Neither age nor lactate dehydrogenase predicted prognosis. No complete responder recurred in the CNS as site of first relapse. CO NCLUSION Despite localized stage at presentation, sinus lymphoma is an aggressive disease, characterized by distant relapse and early mortal ity. Combined-modality therapy with CNS prophylaxis improves outcome c ompared with radiotherapy alone; however, prognosis remains poor. Pati ents with T-lineage disease appear to have a particularly bad outcome. Autologous bone marrow transplantation should be evaluated as first-l ine therapy for those at high risk of relapse.