Document Type

Article

Publication Title

Annals of Translational Medicine

Abstract

Background and objective: Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) are indolent non-Hodgkin lymphomas (NHLs) that can arise in various extranodal organs, often due to chronic inflammation. Gastric MALT lymphomas are well characterized; however, MALT lymphomas may occur in diverse sites such as the ocular adnexa, salivary glands, thyroid, lung, skin, and the small intestine. These site-specific MALT lymphomas have distinct etiologic associations, clinical presentation, and management considerations. This review provides an updated, site-specific overview to guide the diagnostic and therapeutic approach to non-gastric MALT lymphoma in adults.

Methods: We reviewed peer-reviewed articles [2015-2025] on non-gastric MALT lymphomas, focusing on pathophysiology/etiology, diagnostic workup, and treatment strategies for each site. Key sources include recent reviews and guidelines from high-impact journals.

Key content and findings: Chronic antigenic stimulation is a unifying theme in MALT lymphoma genesis, either through infectious etiologies or autoimmune conditions. Ocular adnexal MALT lymphoma (OAML) is linked to Chlamydia psittaci (C. psittaci) infection in certain regions; salivary MALT lymphoma is related to Sjogren's syndrome (SS); thyroid MALT lymphoma develops in the background of Hashimoto's thyroiditis; pulmonary MALT lymphoma can be linked to chronic airway inflammation due to Achromobacter xylosoxidans (A. xylosoxidans); small intestinal MALT lymphoma can be associated with Campylobacter jejuni (C. jejuni) infection; and cutaneous MALT lymphoma can be linked to Borrelia burgdorferi (B. burgdorferi) infection. Diagnostic evaluation requires adequate tissue biopsy for histopathology and immunohistochemistry. MALT lymphomas exhibit an immunophenotype consistent with CD20+, CD79a+, IgM+ with light chain restriction, BCL2+, and negative for CD5, CD10, and cyclin D1. Staging for OAML and cutaneous lymphomas is tumor-node-metastasis (TNM)-based, while the others utilize an Ann Arbor staging system. Computed tomography (CT)/magnetic resonance imaging (MRI) and positron emission tomography (PET)/CT are used to determine the staging and spread of tumors. Treatments are not standardized but consist of therapy with radiotherapy and surgical excision for localized disease, and chemotherapy/for disseminated disease. Intestinal MALT lymphoma differs, as first-line treatment consists of antibiotics and then chemotherapy/immunotherapy.

Conclusions: The non-gastric MALT lymphomas have an excellent prognosis as a whole; relapses are common but manageable, and disseminated disease is rare. Long-term follow-up is recommended in all cases.

DOI

10.21037/atm-25-114

Publication Date

12-31-2025

Keywords

Hashimoto thyroiditis, Mucosa-associated lymphoid tissue lymphoma (MALT lymphoma), Sjogren’s syndrome (SS), marginal zone lymphoma, non-gastric MALT lymphoma

ISSN

2305-5847

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