Multifocal extranodal mucosa-associated lymphoid tissue lymphoma affecting the larynx

Extranodal marginal B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) accounts for about 7% to 8% of all B-cell lymphomas and 50% of all gastric lymphomas. Long-term localized growth is typical of MALT lymphoma. Multifocal manifestations are possible in advanced stages. MALT lymphoma of the larynx is a very rare disease; only 15 cases have been reported in the literature.

We report a case of multifocal MALT lymphoma affecting the larynx associated with extraesophageal reflux, chronic laryngitis, and gastric Helicobacter pylori infection. The staging revealed a recurrent tumor of MALT lymphoma in the stomach and an involvement of the right conjunctiva.

Following recent reports on successful treatment of MALT lymphoma with antibiotics, initial empirical therapy with doxycycline calcium led to a subjective clinical symptom improvement but no objective response as assessed by laryngoscopy, magnetic resonance imaging of the larynx, and esophagogastroduodenal endoscopy. Because of the advanced stage and multiple extranodal manifestations of the MALT lymphoma, the patient received 3 cycles of chemoimmunotherapy according to the FCR protocol (fludarabine phosphate-cyclophosphamide-rituximab). No evidence of disease was observed after a 6-month follow-up.

In the rare diagnosis of MALT lymphoma of the larynx, comprehensive staging is indispensable to exclude multifocal involvement. In contrast to the treatment of primarily localized MALT lymphoma, multifocal disease warrants systemic therapy.