Epidemiology, diagnosis, and treatment of primary pulmonary mucosa-associated lymphoid tissue lymphoma

Introduction

Primary pulmonary lymphoproliferative disorders (PLDs) are a heterogeneous group of uncommon entities (1,2), that encompass both reactive and neoplastic conditions affecting the lung (1-14) (Table 1). Unlike secondary pulmonary involvement by systemic lymphomas or leukemias, primary pulmonary lymphoid proliferations are rare and often diagnostically challenging. They may range from benign hyperplastic lesions, such as nodular lymphoid hyperplasia or follicular bronchiolitis, to aggressive lymphomas, including diffuse large B-cell lymphoma (DLBCL) and lymphomatoid granulomatosis (1,2). Within this spectrum, pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma is the most frequent primary pulmonary lymphoma, accounting for more than 80% of cases (1).

Pulmonary MALT lymphoma

Epidemiology

Pulmonary MALT lymphomas represent only 0.5–1% of all pulmonary malignancies and about 3.6% of all extranodal lymphomas. Among lymphoproliferative disorders (LPDs), they account for approximately 90% of cases (15). They most commonly arise in the sixth to seventh decades of life, with no clear sex predilection. Autoimmune disorders are present in approximately 20% of cases, while smoking is associated in 35–45% of patients (16). A history of chronic infection or inflammation, such as tuberculosis, asthma, chronic obstructive pulmonary disease (COPD) is frequently reported, too. These conditions may promote the development of acquired bronchus-associated lymphoid tissue (BALT), which is considered the substrate for lymphomagenesis in the lung. However, no specific infection or inflammatory disease has been consistently identified as a definitive causal factor. The baseline characteristics of patients with pulmonary MALT lymphoma reported in retrospective series worldwide are summarized in Table 2 (16-25).

Biology and pathogenesis of pulmonary MALT lymphoma

Lymphoid tissue is extremely difficult to find in healthy adults. Lymphoid aggregates can be occasionally observed in children, adolescents, or in the context of chronic lung disease (26). The discovery of BALT in animal models during the 1970s provided the conceptual foundation for understanding pulmonary lymphomagenesis (27). BALT represents an inducible form of MALT, appearing in response to antigenic stimulation and local cytokine release (26,28). While protective in acute infections, persistent stimulation may foster chronic inflammation and eventually promote chronic inflammation and malignant transformation.

The concept of MALT lymphomas was first introduced by Isaacson and Wright in 1983, when they observed gastric lymphomas with histologic features resembling Peyer’s patches (29). This insight led to the recognition of MALT lymphomas as a distinct group of extranodal B-cell neoplasms.

Chronic infections and autoimmune conditions are strongly associated with the development of marginal zone lymphomas (MZLs). In particular, persistent Helicobacter pylori infection is a well-recognized predisposing factor for gastric MZL, while other microorganisms have been implicated in site-specific extranodal MZLs (30-32).

As mentioned before, chronic inflammation represents the etiopathogenethic background of MALT lymphoma of the lung and the causes can be variable. Several infections, including Mycobacteria (33), Trypanosoma whipplei (34), Chlamydiae species, Mycoplasma (35), and Achromobacter xylosoxidans (36) has been linked to pulmonary MALT lymphoma, although the evidence remains inconclusive since criteria for a causal relationship are incompletely fulfilled and less stringent than in the case of Helicobacter pylori and gastric lymphoma (37). In addition, there are reports of pulmonary MALT lymphomas arising as a progression from lymphocytic interstitial pneumonia (38-40). Moreover, autoimmune diseases such as systemic lupus erythematosus, multiple sclerosis, Hashimoto’s thyroiditis, and Sjögren’s syndrome are recognized risk factors for pulmonary MALT lymphoma (41).

The development of MZL is thought to involve a multistep process, beginning with the expansion of normal marginal zone B cells under conditions of chronic antigenic stimulation. Organ-specific inflammation drives persistent immune responses, favoring the proliferation of B-cell clones with reactive B-cell receptors (BCRs) within a supportive microenvironment. Over time, lymphoma progression is sustained by the acquisition of molecular lesions in pathways initially activated by the inflammatory milieu (7). These genetic events deregulate signaling cascades, ultimately allowing the tumor to grow independently of the microenvironment. Although the specific genetic changes vary by site, they commonly converge on pathways critical to B-cell homeostasis, including BCR signaling, nuclear factor-κB (NF-κB), and NOTCH (31).

Chromosomal translocations involving the MALT1 gene are among the most significant genetic alterations in pulmonary MALT lymphoma. The translocation t(11;18)(q21;q21) is particularly frequent and results in constitutive activation of NF-κB signaling, promoting cell survival and proliferation. Less common rearrangements include t(14;18)(q32;q21), also involving MALT1, as well as translocations affecting BCL10 and FOXP1. In addition to translocations, numerical chromosomal abnormalities such as trisomy 3 and trisomy 18 are frequently observed. Point mutations further contribute to lymphomagenesis. Genes such as KMT2D, TNFAIP3, and NOTCH1—which regulate chromatin remodeling, inflammatory responses, and cell signaling—are often disrupted, thereby fostering unchecked growth and survival (42,43).

Taken together, these genetic alterations not only distinguish MALT lymphoma from reactive lymphoid proliferations but also have prognostic and therapeutic implications. Certain molecular profiles may predict resistance to conventional therapies, while others may highlight potential susceptibility to targeted approaches (42,43).

Histopathology and immunophenotype

Pulmonary MALT lymphoma is characterized by dense infiltrates of small atypical lymphoid cells, predominantly localized in the marginal zones around reactive follicles. These infiltrates often extend into the alveolar septa and bronchiolar epithelium, where they form lymphoepithelial lesions, the hallmark of MALT lymphomas. In some cases, the neoplastic B cells show plasmacytic differentiation, and follicular colonization may also be present. Rarely, cells with features resembling centroblasts appear, introducing some cytologic variation into an otherwise monotonous lymphoid population (42).

Immunohistochemically, tumor cells typically express CD20 and surface immunoglobulin, most often of the IgM isotype. A defining feature is light-chain restriction (either kappa or lambda), confirming clonality. These cells are usually negative for CD5, CD10, and cyclin D1, which helps distinguish them from mantle cell lymphoma and follicular lymphoma (7).

The Ki-67 proliferative index is generally low, consistent with the indolent nature of the disease. A background population of reactive T lymphocytes is frequently interspersed among the neoplastic cells. To support the assessment of B-cell clonality and assist in the diagnostic workup, ancillary studies such as fluorescence in situ hybridization (FISH) for MALT1-related chromosomal abnormalities and polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain (IgH) gene rearrangements may be performed. However, these findings are not diagnostic per se and must be interpreted in the context of morphologic, immunophenotypic, and clinical features (42).

Diagnosis and radiological manifestations

Definitive diagnosis of pulmonary MALT lymphoma requires a tissue biopsy, with histopathologic evaluation supported by immunohistochemistry, clonality studies, and FISH for t(11;18) when indicated (7). As pulmonary MALT lymphomas are typically localized to the lung parenchyma, cytology from bronchoalveolar lavage is usually negative, underscoring the need for tissue sampling as the diagnostic gold standard.

Appropriate imaging is important to guide the diagnostic process. Computed tomography (CT) and fluorodeoxyglucose positron emission tomography (FDG-PET) scans play a central role in staging and characterization. The most common radiologic patterns include a pneumonia-like consolidative pattern, a solitary nodular or mass-like pattern, and multiple nodular or mass-like lesions. Hilar lymphadenopathy is observed in up to 30% of cases, while mild pleural effusion occurs in approximately 10% (20). FDG-PET shows high sensitivity in this indolent disease, although FDG avidity in these patients is variable, reflecting differences in baseline metabolic activity (7,44). Most patients are diagnosed at stage I, according to the Ann Arbor staging system.

Table 3 summarizes the diagnostic workup at the time of diagnosis and relapse.

Clinical course and prognosis

Pulmonary MALT lymphomas typically have an indolent course, often remaining confined to the lung for prolonged time before disseminating. Even when lymph nodes are involved, the disease generally shows limited aggressiveness (16).

The clinical presentation is variable. Nearly half of patients are asymptomatic at diagnosis, with the disease detected incidentally during investigations prompted by an abnormal chest radiograph. When symptoms occur, they are usually nonspecific, including dyspnea, cough, chest pain, or hemoptysis. B-symptoms are extremely rare and tend to be associated with more aggressive disease variants. Laboratory abnormalities such as anemia and/or thrombocytopenia may occasionally be observed.

The disease course is usually very indolent, with 5- and 10-year overall survival (OS) rates of approximately 85–90% and 70–90%, respectively, as reported in recent retrospective series from individual centers and cooperative groups (16,18-22,45). Although analyses from the Surveillance, Epidemiology, and End Results (SEER) cancer registries reported somewhat less favorable outcomes, with 5- and 10-year OS rates of 75% and 53%, respectively, the cause-specific survival in this report was 86% and 75% at 5 and 10 years (46). In patients with primary pulmonary MALT lymphoma receiving frontline systemic therapy (rituximab and/or chlorambucil) in a randomized clinical trial (47), the OS was 78% at 5 years and 61% at 10 years (Figure 1). Discrepancies in survival data across the literature underscore the influence of different data sources (e.g., tumor registries, referral center databases, and clinical trials). Furthermore, they highlight potential biases arising from variations in patient age, comorbidities, disease stage, treatment type and treatment era, and the distribution of prognostic features across studies.

Figure 1 OS probability in patients with MALT lymphoma of the lung [adapted from Zucca et al., J Clin Oncol, 2017 (47)]. MALT, mucosa-associated lymphoid tissue; OS, overall survival.

Local recurrence is not uncommon. Importantly, advanced clinical stage at diagnosis correlates with inferior progression-free survival (PFS), though it does not necessarily translate into reduced OS (16). On the other hand, Joffe et al. showed that, in a retrospective study of 123 patients, cases treated with surgery achieved a superior event-free survival (EFS) compared with those managed with active surveillance or systemic treatment (6-year EFS: 74% vs. 65% vs. 62%, respectively; P=0.013) while all treatment groups had excellent OS at 6 years (93%) (19).

Treatment strategies

The management of pulmonary MALT lymphoma should be individualized based on disease stage, symptom burden, comorbidities, and patient preferences. A variety of strategies have been employed, including surgical, radiotherapeutic, and systemic pharmacologic approaches (48). However, data specific to the management of pulmonary MALT lymphoma remain limited. Most clinical trials in this field have focused on indolent non-Hodgkin lymphomas (NHLs), predominantly enrolling patients with follicular lymphoma, while MZL subtypes are usually grouped together, often with only a small number of cases per subtype. As a result, therapeutic decisions in pulmonary MALT lymphoma are extrapolated mainly from studies in broader MZL populations or in indolent NHLs in general. Table 3 summarizes treatment strategies in pulmonary MALT lymphoma.

In asymptomatic patients, active surveillance with periodic imaging is often appropriate, given the typically indolent course of the disease (19). An expectant strategy may also be considered in cases where radical surgery resection was part of the diagnostic process (49).

For localized pulmonary MALT lymphoma, radiotherapy (RT) remains the cornerstone of management. The conventional regimen involves the delivery of 24 Gy, but interest has grown in low-dose approaches aimed at maintaining efficacy while reducing toxicity and treatment burden. The FoRT study compared 4 Gy with 24 Gy in patients with follicular lymphoma or MZL, reporting a median time to local progression of 12.3 vs. 19.3 months and a 5-year PFS of 70% vs. 90% (P<0.0001), with no significant difference in OS (50). These findings suggest that as many as two thirds of patients treated with 24 Gy may be overtreated. Consequently, very low-dose RT (VLDRT) regimens, particularly the 2×2 Gy scheme, are increasingly being explored with encouraging results. Wu et al. and Freret et al. reported a 100% overall response rate (ORR) in patients with pulmonary MALT; 67–95% of patients achieved a complete response (CR), with durable local control and minimal toxicity (51,52). Thus, VLDRT is increasingly recognized 229 as a feasible and patient-friendly alternative for indolent pulmonary MALT lymphoma, particularly in elderly or comorbid populations. In the same setting, surgical resection, although not generally recommended in lymphomas, may be considered an appropriate approach (48).

For patients with advanced or symptomatic disease, systemic therapy is required. Rituximab-based regimens represent the standard of care, despite the lack of prospective studies conducted specifically in this patient population. The IELSG19 randomized trial of MALT lymphomas, which included 42 patients with primary lung localization, demonstrated the synergistic effect of rituximab combined with chlorambucil, confirming the superiority of immunochemotherapy over monotherapy (47). Subsequent studies established bendamustine-rituximab (BR) as the preferred regimen in advanced disease, however, the number of enrolled patients with lung lymphoma was not reported (53,54).

The GELTAMO phase II study evaluated frontline BR with response-adapted de-escalation in patients with MALT lymphoma. Patients achieving complete remission after three cycles received only four cycles total, while those in partial remission continued to six cycles. The study reported a CR rate of 98%, with 7-year PFS and OS of 93% and 96.5%, respectively (53). Similarly, Alderuccio et al. analyzed 237 patients in a US-Italy retrospective study. With a median of six cycles of BR, ORR reached 93% and CR 81%. Five-year PFS and OS were 81% and 90%, respectively (54).

Rituximab maintenance is not routinely recommended. In the IELSG38 study, maintenance appeared to facilitate long-term disease control and was associated with improvements in EFS and PFS, but the benefit was not sufficient to establish it as standard practice (55).

In the relapsed or refractory setting, there is no single accepted standard of care. Treatment should be individualized, with the goals of alleviating symptoms, restoring normal blood counts, and improving quality of life, while avoiding overtreatment and excess toxicity. Active surveillance remains appropriate for asymptomatic patients, whereas involved-site RT may be considered for localized symptomatic relapse. Finally, novel targeted agents are expanding the landscape of systemic therapy (48) although no prospective studies have specifically evaluated the use of these agents in patients with primary pulmonary MALT lymphoma. The available data are therefore derived from studies conducted in patients with MZLs at other sites. Bruton’s tyrosine kinase (BTK) inhibitors have demonstrated promising efficacy with a favorable safety profile, leading to zanubrutinib approval in several countries (56). Lenalidomide, an immunomodulatory agent, may also provide a useful alternative option to chemotherapy (57-59). Novel agents, including bispecific antibodies, antibody-drug conjugates, and BCL2 inhibitors, are currently under investigation (48). Across all therapeutic modalities, the choice of treatment should carefully consider the expected toxicity profile in addition to efficacy. RT may cause pulmonary inflammation and fibrosis, though very low-dose regimens significantly limit these risks. Immunochemotherapy, including BR, is associated with myelosuppression, immunosuppression, and gastrointestinal side effects, while BTK inhibitors carry risks of cardiovascular events, bleeding, and infections. Importantly, these adverse events generally reflect those observed in the treatment of other indolent lymphomas, and modern strategies, such as low-dose RT and response-adapted chemotherapy, serve to minimize toxicity while maintaining therapeutic effectiveness.

Conclusions

Pulmonary MALT lymphoma is a rare but distinct clinicopathological entity characterized by antigen-driven pathogenesis, peculiar genetic alterations, indolent natural course, and excellent prognosis. Most patients present with localized disease, where RT including VLDRT, offers durable control with minimal toxicity. In advanced cases, rituximab-based immunochemotherapy, particularly BR, provides high response rates and long-term disease control.

Targeted therapies, especially BTK inhibitors, such as zanubrutinib, are expanding the therapeutic armamentarium, with ongoing clinical trials seeking to refine management further.

Acknowledgments

We would like to thank Rita Gianascio Gianocca for the secretarial help.

Footnote

Peer Review File: Available at https://cco.amegroups.com/article/view/10.21037/cco-2025-1-134/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://cco.amegroups.com/article/view/10.21037/cco-2025-1-134/coif). The authors have no conflicts of interest to declare.

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