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Pulmonary marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) are disorders comprising less than 1% of lung malignancies and non-Hodgkin lymphomas. Pulmonary MALT lymphoma is an indolent disease with the majority of patients presenting at an early stage.
A definitive diagnosis requires histologic evaluation of tissue obtained via image-guided needle biopsy or minimally invasive surgical biopsy. Image-guided biopsies often are inadequate with a diagnostic specimen requiring a surgical biopsy.
Current therapy options include surgical resection, chemotherapy, and targeted therapy such as rituximab. The results of surgical resection for early-stage pulmonary MALT (stage IE, IIE) are conflicting. There have been some studies supporting its use with improved outcomes and a prolonged disease-free survival,
review 51 patients with pulmonary MALT lymphoma to shed further light on this disorder. The authors find that given the indolent nature of the early-stage pulmonary MALT lymphomas, surgery alone may be the optimal therapy. The authors further suggest that sublobar resections that offer lung volume preservation and fewer complications may provide equivalent outcomes to lobectomy for peripheral lesions, although the limited sample size of the study makes it hard to make definitive conclusions. Because many patients are first diagnosed by surgical biopsy, the authors further argue that resection can serve not only as a means of diagnosis but also as first-line therapy in those with early-stage disease.
Randomized trials are the obvious gold standard but near impossible to complete with sufficient power for rare disorders. How then are we to obtain meaningful data to base treatment? To this end, rare diseases such as pulmonary MALT lymphomas will benefit from having shared international registries to allow pooling of data regarding the natural history of disease and the response to treatment to allow generation of treatment guidelines. Until then, the cut may be the cure for early-stage pulmonary MALT (Figure 1).
A retrospective international study on primary extranodal marginal zone lymphoma of the lung (BALT lymphoma) on behalf of International Extranodal Lymphoma Study Group (IELSG).