Lung Cancer: Student/Resident Case Report Poster - Lung Cancer I |

Primary Pulmonary Diffuse B Cell Lymphoma Mimicking Cryptogenic Organizing Pneumonia FREE TO VIEW

Laura Barnes, MD; Eric Scholten, MD; Anas Khalil, MD; Samir Makani, MD; Rebecca Sell, MD
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University of California San Diego, San Diego, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):752A. doi:10.1016/j.chest.2016.08.847
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SESSION TITLE: Student/Resident Case Report Poster - Lung Cancer I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary Pulmonary Lymphoma (PLL) is an uncommon type of Non-Hodgkins Lymphoma (NHL). Primary Pulmonary Lymphoma - Diffuse Large B-cell (PPL-DLBCL) is even more rare, comprising <5% of all NHL1. Here we present a case of PPL-DLBCL diagnosed on autopsy and discuss the diagnosis of this rare disease and its association with organizing pneumonia.

CASE PRESENTATION: A 66 year-old man with hepatitis C treated with ledipasvir/sofosbuvir presented to clinic with two weeks of dry cough and dyspnea. CT showed scattered nodules surrounded by ground glass and few sub-centimeter lymph nodes. Biopsy of nodules showed inflammation without features of malignancy. Rheumatological markers and fungal serologies were negative. At five months, repeat CT demonstrated resolution of some nodules, enlargement of others, and the development of new nodules. Transbronchial biopsies yielded tissue consistent with organizing pneumonia, however despite several months of steroids his symptoms progressed. Repeat transbronchial biopsies and Bronchoalveolar Lavage (BAL) showed nonspecific inflammation and so a surgical lung biopsy was planned. Unfortunately, prior to surgery he developed acute respiratory failure and expired. Post-mortem lung biopsy demonstrated the nodules were DLBCL, germinal center phenotype.

DISCUSSION: Organizing pneumonia is associated with many different exposures and disease processes, and has rarely been associated with lymphoma. There are several case reports of organizing pneumonia in the setting of PPL and chemotherapy, and a few case reports noting organizing pneumonia as the initial presentation of PPL2. PPL-DLBCL is rare and diagnosis can be challenging due to nonspecific presenting symptoms, and often an absence of typical B symptoms. CT imaging is variable, including single or multiple lung nodules, cavitary masses, infiltrates and consolidation. The yield of endobronchial biopsies is only 10-30%3. Diagnosis by video-assisted thorascopic (VATS) or open wedge resection is more accurate, however these are invasive and typically not first line diagnostic modalities. BAL is typically low yield. PPL-DLBCL can be aggressive but is usually responsive to conventional lymphoma chemotherapies. Germinal center phenotype conveys the worst prognosis.

CONCLUSIONS: PPL-DLBCL is rare and challenging to diagnose but has a good prognosis with chemotherapy, making early identification key. In patients with atypical presentations of organizing pneumonia or who don’t respond to steroids, PPL should be considered. Diagnosis often requires a surgical lung biopsy.

Reference #1: Parissis H. Forty years literature review of primary lung lymphoma. J Cardiothoracic Surg. 2011;6:23.

Reference #2: Safadi R, Berkman N, Haviv YS et al. Primary non-Hodgkin's lymphoma of the lung presenting as bronchiolitis obliterans organizing pneumonia. Leuk Lymphoma. 1997 Dec;28(1-2):209-13.

Reference #3: Kim JH, Lee SH, Park J et al. Primary Pulmonary Non-Hodgkin's Lymphoma. Jpn. J. Clin. Oncol. (2004) 34 (9): 510-514.

DISCLOSURE: The following authors have nothing to disclose: Laura Barnes, Eric Scholten, Anas Khalil, Samir Makani, Rebecca Sell

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