Lung Cancer |

Recurrence of Extranodal Marginal Zone B-Cell Lymphoma of Bronchus-Associated Lymphoid Tissue Presenting as Superior Vena Cava Syndrome FREE TO VIEW

Larysa Gromko, MD; Narjust Perez-Florez, MD; Tatyana Feldman, MD
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Rutgers New Jersey Medical School, Newark, NJ

Chest. 2014;146(4_MeetingAbstracts):643A. doi:10.1378/chest.1995170
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

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

INTRODUCTION: Primary pulmonary lymphoma is a rare clinical entity, accounting for 0.5-1% of pulmonary malignancies and less than 1% of all lymphomas. Extranodal marginal zone lymphoma (EMZL) of bronchial-associated lymphoid tissue (BALT) is a distinct subtype of low-grade B-cell lymphomas and makes up the majority of primary pulmonary lymphomas.

CASE PRESENTATION: A 64-year-old-man with 120 pack-years of smoking and history of right upper lobe pulmonary EMZL status post complete surgical resection four years prior, presented with a six month history of shortness of breath, orthopnea, voice changes, and dysphagia. He denied any weight loss, fevers, or night sweats. On physical examination, he was found to have neck and periorbital edema as well as facial plethora. CT imaging revealed a large mass extending from the right superior mediastinum into the right upper lung measuring 11.4 x 11.7 cm with superior vena caval compression and tracheal deviation, as well as a spiculated 5.4 x 3.1 cm mass-like opacity in the posterior right lower lobe. Transbronchial biopsy of the right upper lung mass revealed atypical lymphoid B-cells consistent with extranodal marginal zone BALT lymphoma. Immunohistochemical staining showed atypical cells to be CD20+, BCL2+, with Ki67 proliferation of 20%. Chemotherapy with bendamustine plus rituximab was initiated and the patient achieved complete response after six cycles. No relapse has been observed in a 3 year follow up period.

DISCUSSION: BALT lymphomas are thought to be associated with chronic antigenic stimulation including smoking, autoimmune diseases, and inflammatory disorders. BALT lymphomas follow an indolent course and most commonly, patients are asymptomatic and lesions are discovered incidentally on imaging, although nonspecific pulmonary symptoms may be present. Radiographic findings of BALT lymphoma range from single to multiple nodules, masses or infiltrates. Although BALT lymphoma has a favorable prognosis, the disease tends to relapse. A generalized treatment approach has not been well established, but recent clinical trials with bendamustine plus rituximab have demonstrated prolonged disease free survival.

CONCLUSIONS: Massive lung infiltration of BALT lymphoma is less commonly described and we find the extent of disease involvement resulting in superior vena cava syndrome in our case to be a unique presentation of a rare lymphoma.

Reference #1: Borie, R. et al. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J 2009; 34: 1408-16.

Reference #2: Oh, S.Y. et al. Pulmonary marginal zone B-cell lymphoma of MALT type—What is a prognostic factor and which is the optimal treatment, operation, or chemotherapy?: Consortium for Improving Survival of Lymphoma (CISL) Study. Ann Hematol 2010. 89(6):563-8.

Reference #3: Salar, A. et al. Final Results of a Multicenter Phase II Trial with Bendamustine and Rituximab as First Line Treatment for Patients with MALT lymphoma (MALT-2008-01). Blood 2012. 120: Abstract 3691.

DISCLOSURE: The following authors have nothing to disclose: Larysa Gromko, Narjust Perez-Florez, Tatyana Feldman

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