Abstract Introduction Accounting for approximately eight percent of lymphomas, marginal zone lymphoma (MZL) is an indolent B-cell non-Hodgkin lymphoma thought to originate from maturing B-cells in the marginal zone of lymphoid follicles. Over half of MZL cases are extranodal, in mucosal tissues, giving the name mucosa-associated lymphoid tissue lymphoma. We present a patient found to have two endobronchial lesions diagnosed as pulmonary extranodal MZL. Case Report A 65-year-old female with interstitial lung disease and renal cell carcinoma treated with right radical nephrectomy was found to have a spiculated right upper lobe (RUL) nodule and enlarging right lower lobe (RLL) mass on chest CT. RLL wedge resection and mediastinal lymph node sampling found chronic inflammation, lymphoid aggregates, and peribronchiole metaplasia. She underwent surveillance imaging biannually with Oncology. A current non-contrasted chest CT showed enlargement of the RLL posterior lesion measuring 4.5x3.0cm, a new 1.1cm RUL spiculated lesion, and enlarged mediastinal lymph nodes, which demonstrated hypermetabolic activity on Positron Emission Tomography-CT (PET/CT) scan. She underwent diagnostic bronchoscopy which demonstrated a highly vascular anterior midline tracheal mass (Figure 1A), as well as hypertrophic mucosal changes in the bronchus intermedius (Figure 1B). There was also extrinsic compression of the right middle lobe orifice, preventing visualization distally (Figure 1C). Transbronchial biopsies of the RLL mass and RUL nodule were non-diagnostic. Biopsies from mediastinal and hilar lymph nodes found no malignancy. Biopsies from the tracheal mass and bronchus intermedius were obtained due to the finding of endobronchial abnormalities. Histopathology revealed mature B-cell non-Hodgkin lymphoma with plasmacytic differentiation, with further differentiation, via morphologic and immunophenotypic studies, into extranodal pulmonary MZL, without large cell transformation. Oncology initiated treatment with Bendamustine and Rituximab. Discussion Approximately half of pulmonary extranodal MZL are asymptomatic when diagnosed. While biopsy is the gold standard, acquiring sufficient tissue with structure intact often proves difficult. Imaging findings are quite variable: the most commonly noted lesions come in segmental or lobar constellations, though multifocal nodules and discreet masses also are seen. Nonspecific ground-glass opacities and exudative pleural effusions may coexist, but endobronchial lesions are only rarely reported. A high degree of suspicion should be maintained regarding nonspecific imaging findings, and thorough examination of central airways can aid diagnosis. This abstract is funded by: None
Springer et al. (Fri,) studied this question.