BackgroundThe occurrence of primary lymphoma in the lung is exceedingly uncommon, with the majority of these cases representing marginal zone lymphoma originating from bronchus-associated lymphoid tissue (BALT). BALT lymphoma (BALToma) is a rare and indolent type of lymphoma. Given the limited number of cases and the necessity for prolonged observation, treatment options based on lung disease status remain uncertain.
Methods This retrospective study aimed to investigate the characteristics and survival outcomes of patients with marginal zone lymphoma (MZL) involving the lung at Samsung Medical Center diagnosed between 2003 and 2019. The disease status was classified as unilateral lung disease, bilateral lung disease, or lung disease with other site involvement. The treatment methods were classified into four categories: observation and waiting, surgical intervention, chemotherapy, and a combination of surgery and chemotherapy, followed by radiotherapy. Progression-free survival (PFS) and overall survival (OS) were subsequently compared using Kaplan-Meier survival analysis.
Results A total of 40 patients were analyzed, and the median OS was not reached, with a median PFS of 156 months. The 10-year OS rate was 89.1%, and the 10-year PFS rate was 55.1%. Of the 40 patients, 14 had unilateral stage 1 disease and 26 had stage 4 disease. Of these, 12 patients exhibited bilateral lung disease, while 14 patients demonstrated involvement of other sites. In patients with unilateral lung disease, the median PFS for those who underwent surgery was not reached and was significantly improved compared to those who did not undergo surgery, with a median PFS of 37.4 months (p-value = 0.00465). In patients with bilateral lung disease, the median PFS was not reached in the chemotherapy group, in comparison to 26.3 months in the non-chemotherapy group. This represents a significant increase (p-value = 0.00684). In a multivariate analysis based on clinical information, significant decreases in PFS were observed in patients with involvement of other sites, those with air-bronchogram presentation on CT scan compared to patch or nodular lesions, and those aged 70 years or older. No factors were identified that significantly increased OS.
Conclusion In patients with BALToma, the tailoring of treatment strategies based on the disease status of the lung may result in an increased PFS. Surgery plays a pivotal role in the treatment of this condition, particularly in cases of unilateral lung disease, whereas chemotherapy is an effective treatment for bilateral lung disease.
Kim:Kyowa-Kirin: Research Funding; F. Hoffmann-La Roche Ltd: Research Funding; BeiGene: Research Funding; Sanofi: Research Funding; Boryong: Research Funding; Donga: Research Funding.
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