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Lung Cancer |

Primary Pulmonary Marginal Zone B-Cell Lymphoma Without Associated Autoimmune or Infectious Disease

Patrick Smith, DO; Michael Perkins, MD
Author and Funding Information

National Capital Consortium (Walter Reed) Program, Washington, DC


Chest. 2014;146(4_MeetingAbstracts):618A. doi:10.1378/chest.1991519
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Abstract

SESSION TITLE: Cancer Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary pulmonary marginal zone B-cell lymphoma (P-MZL) is rare form of extranodal non-Hodgkin’s lymphoma (NHL), representing less than 1% of all diagnosed NHL and less than 1% of primary lung malignancy. The development of P-MZL is typically associated with prolonged antigenic stimulation from a preceding disease process, either autoimmune, immunodeficiency, or infectious.

CASE PRESENTATION: A 53 year-old male was seen in the emergency room for complaints of chest pain. A screening chest X-ray demonstrated a large opacity (5 cm x 3.3 cm) in the right middle lobe (RML). A subsequent dedicated computed tomographic scan of the chest discovered that the RML opacity was a pulmonary mass (measuring 5.4 cm x 4 cm) with air bronchograms. A review of prior imaging demonstrated the same mass was evident on an electron beam computed tomographic scan performed approximately three years prior, but had now enlarged. Unfortunately, the mass was not addressed at that time. Bronchoscopy was then performed. Histopathology of the mass obtained from a transbronchial revealed monoclonal B-cell proliferation consistent with extranodal marginal zone lymphoma. An extrapulmonic source was not identified via a positron emission tomographic scan, esophagogastroduodenoscopy, or bone marrow biopsy. He was subsequently treated with 6 cycles of bendamustine followed by RML resection.

DISCUSSION: The specific etiology of P-MZL has not been directly identified, but it has been strongly associated with Sjögren syndrome, amyloid deposits, connective tissue disease, H. pylori infection, combined immunodeficiency syndromes, adenocarcinoma, and AIDS. In our patient, an underlying autoimmune or infectious disease process, which is usually responsible for P-MZL, was not identified. Antibody titers against viral hepatitis and HIV were both negative. Gastric tissue samples obtained via EGD were negative for Helicobacter pylori. Serum protein electrophoresis, immunoglobulin panel, and connective tissue disease screening was also negative. Regardless, there is no consensus on management of this rare malignancy

CONCLUSIONS: We present an unusual case of P-MZL that is not associated with any underlying autoimmune disease, immunodeficiency, or chronic infection.

Reference #1: Borie R, Wislez M, Thabut G, et al. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J. 2009;34(6):1408-1416.

Reference #2: Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphoma. Eur Respir J 2002; 20:750-762.

DISCLOSURE: The following authors have nothing to disclose: Patrick Smith, Michael Perkins

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