Extranodal laryngeal MALT lymphoma is an extremely rare cause of laryngeal masses and dysphagia, representing less than 1% of laryngeal neoplasms. It typically presents as an indolent submucosal lesion with nonspecific symptoms such as dysphonia, cough, globus sensation or progressive dysphagia. Histologically, these lymphomas consist of marginal zone B cells with a characteristic CD20+ phenotype and variable plasmacytic differentiation. Lymphoplasmacytic lymphoma (LPL) and plasmacytoma are exceptionally uncommon alternative diagnoses.
A 46-year-old man, smoker of 10 cigarettes/day, presented with mixed high dysphagia for two years with recent progression. Past medical history included asthma and a duodenal ulcer diagnosed 13 years earlier associated with Helicobacter pylori infection, with confirmed eradication. A barium swallow showed a filling defect suggestive of an oesophageal polyp. Upper endoscopy revealed a 3-cm oval, submucosal-appearing lesion arising from the left arytenoid, covered by normal mucosa and without proximal oesophageal extension. The procedure was suspended due to intolerance, preventing gastric inspection. Fibroscopy confirmed a rounded lesion on the left aryepiglottic fold partially occupying the ipsilateral piriform sinus. Cervical computer tomography (CT) demonstrated a solid polypoid mass originating from the same region. A supraglottic partial laryngectomy was performed to obtain a complete diagnostic specimen. Histopathology was suggestive of extranodal marginal zone B-cell lymphoma (MALT type) with plasmacytic differentiation. Molecular studies subsequently demonstrated B-cell clonality, supporting this suspicion, although definitive diagnosis is still pending. The patient is currently undergoing full hematologic work-up, which include repeat upper endoscopy to exclude synchronous gastric MALT involvement.
In contrast to gastric MALT lymphoma, laryngeal MALT is not associated with H. pylori infection. Chronic inflammatory processes are thought to play a more relevant pathogenetic role. Comprehensive staging is essential, particularly upper gastrointestinal endoscopy with systematic gastric biopsies, because synchronous gastric involvement or multifocal disease may alter management. While H. pylori eradication is effective for gastric MALT lymphoma and may induce remission of extragastric manifestations, it is not an effective therapy for isolated laryngeal MALT lymphoma. Instead, involved-site radiotherapy is considered the treatment of choice, providing excellent local control and long-term remission. This case emphasises the value of endoscopic evaluation in patients with persistent dysphagia, not only for intraluminal oesophageal pathology but also for extrinsic or juxta-oesophageal lesions. Laryngeal MALT lymphoma, although rare, should be considered in the differential diagnosis of submucosal laryngeal masses. Complete staging, including gastric endoscopy, is essential to establish disease extent and guide optimal treatment.