Extranodal lymphomas of the head and neck. A 20-year experience.

Background: Extranodal non-Hodgkin lymphoma (NHL) of the head and neck is a relatively uncommon disease. Over the last 3 decades, a variety of systems, including the Rappaport, Luke-Collins, and Working Formulation classifications, have been used to classify extranodal NHLs of the head and neck. Most studies have included a relatively small number of patients, used different modalities of therapy, and did not include all head and neck sites. These limitations make comparisons between different studies and drawing any conclusions difficult.

Objectives: To describe in a uniform fashion a relatively large number of patients with extranodal NHL of the head and neck treated at the same institution, using only the most current classification system and to describe the clinical features, behavior, and outcome of this relatively uncommon, but potentially curable disease.

Design: A retrospective study of 98 patients with extranodal NHL of the head and neck. All patients were reclassified according to the Working Formulation system (regardless of the time of diagnosis) in order to uniformly define the clinical course of this disease in the head and neck.

Setting: A tertiary care referral center.

Results And Conclusions: The sinonasal tract was the most commonly involved site (25%). If the nasopharynx (16%), tonsil (12%), and base of tongue (8%) are grouped together, this combined site (Waldeyer ring) becomes the most common site of disease (36%). Patients with tonsillar lymphoma had a 20% incidence of associated gastrointestinal involvement. Approximately 50% of the patients had associated nodal disease, and only 20% had systemic or B symptoms. Three fourths of the patients had stage I or II disease, and approximately two thirds had intermediate-grade lymphoma. Radiation therapy was the primary modality of therapy for localized disease (stages I and II), especially for low-grade lymphomas. Combination chemotherapy with or without radiation was used for more advanced disease and for intermediate- and high-grade lymphomas. Surgery was limited to establishing the diagnosis. Two thirds of the patients had a remission after initial therapy. Two thirds of these patients had no further relapse. Three fourths of the patients with relapse after initial remission died of their disease. The overall and disease-free survival rates for all patients were 60% and 50%, respectively. Outcome of therapy was related to stage and histologic grade. Patients with lymphomas of high histopathologic grade and recurrent and recurrent and disseminated disease had the poorest prognosis.