Secondary lymphoma of Waldeyer's ring: natural history and association with prior extranodal disease

Seven of 16 patients with secondary lymphoma of Waldeyer's ring (15 with non-Hodgkin's lymphoma and one with Hodgkin's disease) had their primary diagnoses made at another extranodal site, usually the gastrointestinal tract. The median interval until development of Waldeyer's ring disease was 37 months. Presenting features were similar to those in previous reports of primary lymphoma of Waldeyer's ring except for the striking degree of cranial nerve dysfunction (83 per cent) in patients with secondary nasopharyngeal disease. Non-Hodgkin's lymphomas were predominantly diffuse (80 per cent) with large cell morphology (53 per cent). Of nine patients who obtained complete remission after Waldeyer's ring involvement, six relapsed (median, 15 months), whereas three remain in complete remission at eight, 32, and 288 months. Twelve patients died (median length of survival, 20 months), eight of whom had disseminated disease, including one patient who also developed massive thyroid lymphoma. Three patients survived at least six years after involvement of Waldeyer's ring. This study confirms the frequent association of secondary lymphoma of Waldeyer's ring with prior extranodal disease, particularly of the gastrointestinal tract. The long latent period and the prolonged survival seen in several patients suggest that occasionally secondary lymphomas of Waldeyer's ring may represent metachronous primary tumors.