Mantle cell lymphoma involving tonsils: a clinicopathologic study of 83 cases

We report 83 cases of mantle cell lymphoma (MCL) involving the tonsil as initial manifestation (IM). The median age at the time of tonsillar involvement was 58 years (range, 35–79 years). Most (85%) patients presented similar to acute tonsillitis. Lymphadenopathy (84%) and advanced stage of disease (81%) were frequent. With a median follow-up of 6.1 years (range, 0.5–18.4 years), the median overall survival (OS) was 11.3 years for all patients. Cases with classic MCL morphology demonstrated a superior OS (median OS: 11.7 years versus 7.8 years for aggressive morphology, P = 0.0361). Approximately 20% of patients had limited stage of disease, and they had excellent outcomes (median OS: not reached versus 11.3 years for advanced-stage MCL, P = 0.0479). All the patients were alive after a median follow-up of 6.6 years (range, 1–16.2 years). There were no differences in relapse-free survival in morphology and stage (P > 0.05). When tonsils were involved by relapsed MCL, patients less commonly had acute tonsillitis–like symptoms, lymphadenopathy, and advanced stage of disease compared to MCL as IM. Patients in the relapse group had poorer OS than patients in the IM group from the time of tonsillar involvement by MCL to the date of death or last follow-up (7.8 versus 11.7 years, P = 0.003). Compared with a group of 93 patients whose initial biopsy specimen was a lymph node, patients whose initial biopsy specimen was tonsil had similar OS (11.7 versus 8.8 years, P = 0.1764). However, patients with tonsillar MCL more commonly had limited stage disease (19% versus 8%, P = 0.0385) and a low-risk Mantle Cell Lymphoma International Prognostic Index score (71% versus 47%, P = 0.0025).