Primary lymphoma of Waldeyer's ring. Clinicopathologic study of 68 cases

The clinical and pathologic findings were studied in 68 patients with primary lymphoma of Waldeyer's ring (WR). The initial sites included: tonsil (51%; 9% bilateral), nasopharynx (35%), base of tongue (9%), and multiple areas (4%). The mean age was 58 years, with a male to female ratio of 1.1:1.0. Tonsillar and base of tongue lesions presented with sore throat and dysphagia, whereas nasopharyngeal lymphomas most commonly presented with nasal, auditory, and cranial nerve sympatomatology. A neck mass was the sole presenting symptom in 12% of patients. Work-up showed 42% Stage I, 47% Stage II, 4% Stage III, and 7% Stage IV. All were non-Hodgkin's lymphomas. Diffuse architecture predominated (71%) and 70% had a histiocytic or large cell morphology (diffuse histiocytic 51%, nodular histiocytic 19%). The National Cancer Institute (NCI) Working Formulation grades were 13% low, 81% intermediate, and 6% high. In difficult cases, detection of monoclonal immunoglobulin, absence of keratin staining, and lack of epithelial features by electron microscopy were useful adjuncts aiding in diagnosis. Following complete remission, 28 patients relapsed, 75% of these within 2 years (median, 10 months). Thirty-seven (54%) patients died with disease (median survival, 27 months). Of these, 89% had disseminated lymphoma. Local-regional disease was the direct cause of death in 13%. Recurrence or persistence of disease in WR occurred in 9% of cases, most with initial clinical evidence of soft tissue extension. Stage I patients had a significantly better median survival (67 months) than Stage II patients (20 months) (P = 0.03). Prognostically favorable histologic parameters included lower NCI Working Formulation grade and follicular architecture. Ten patients (15%) developed extranodal disease, eight involving the gastrointestinal (GI) tract, and all died within 1 year of its occurrence. The findings indicate that stage and certain histologic parameters are important prognostic factors in WR lymphoma. The study confirms the association of primary WR lymphoma with other extranodal disease, particularly involving the GI tract, and emphasizes the poor prognosis of patients in whom this occurs.