Nasal-paranasal oropharyngeal (NPOP) non-Hodgkin's lymphoma (NHL) is a disease of the very young (median age, 5 years) and of the aging adult (median age, 50-60 years). Of a total of 208 pediatric patients with NHL studied, 20 (9.6%) had primary NPOP. Sixty percent of the patients had Stage I and I1 disease. Primary sites were maxillary sinus in eight patients; tonsils in eight; posterior pharynx in two; mandible in one; and orbit in one patient. Histologically, the disease is different than that of the adults since most patients had B-cell lymphomas of the diffuse undifferentiated type (Rappaport) or small cell non-cleaved types (Lukes-Collins, Kiel, and Working Formulation). None of these patients had gastrointestinal involvement. All patients were treated with the LSA,-L, regimen and radiation therapy was given to primary unresectable tumors and regional metastases. The lymphoma event-free survival was 75%, with a median observation period of 99+ months. In staging systems that refer mostly to amount of disease outside of the primary (such as ours, Murphy's, and the Ann Arbor staging systems) stage did not correlate well with disease-free survival. In the TNM staging of 1977, a staging system that refers to size of primary tumor as well as regional and systemic disease, stage correlated better with prognosis and survival. In our staging system, eight of 12 patients (66.7%) with Stage I and 11 disease; four of four with Stage 111; two of two with Stage IVA; and zero of two with Stage IVB survived. In the TNM staging system, three of three patients with Stage I1 and I11 disease and 12 of 18 patients (67%) with Stage IV disease survived. All recurrences occurred early suggesting that early intensification of chemotherapy may produce better results. Cancer 65:1438-1444,1990.
RIMARY NASAL, paranasal, oral, and pharyngeal P (NPOP) lymphoma has been called a disease of advancing age in adults.' In most reports, only a few pediatric cases are mentioned and these are often included in the analysis of adult patients. We believe that pediatric NPOP lymphoma is a distinct entity among childhood lympho-From the