Authors:
Brandon Jakubowski, Rosechelle M. Ruggiero
Abstract:
TOPIC: Lung Cancer TYPE: Fellow Case Reports INTRODUCTION: Mucosal-associated lymphoid tissue (MALT), is a system of lymphoid tissue found within various organs that plays a role in regulating local immunity. As this tissue contains T, B, and plasma cells, it is the potential site for the development of lymphoma. MALT lymphomas are a form of B-cell extranodal lymphoma that can originate from marginal zone lymphocytes within the lung, referred to as pulmonary marginal zone lymphoma (PMZL). CASE PRESENTATION: We present a 42-year-old female refer (...)
TOPIC: Lung Cancer TYPE: Fellow Case Reports INTRODUCTION: Mucosal-associated lymphoid tissue (MALT), is a system of lymphoid tissue found within various organs that plays a role in regulating local immunity. As this tissue contains T, B, and plasma cells, it is the potential site for the development of lymphoma. MALT lymphomas are a form of B-cell extranodal lymphoma that can originate from marginal zone lymphocytes within the lung, referred to as pulmonary marginal zone lymphoma (PMZL). CASE PRESENTATION: We present a 42-year-old female referred to our clinic for evaluation of pulmonary nodules and mediastinal adenopathy. She had a one year history of Sjogren's syndrome. At that time she had no pulmonary or B symptoms. To evaluate her mediastinal adenopathy, she was referred for endobronchial ultrasound where pathologic evaluation revealed only a polymorphous lymphoid population and was negative for malignancy. She had several endobronchial hypervascular and polypoid nodules within the mainstem airways. Pathology from these nodules demonstrated an atypical lymphoid infiltrate, but was unable to be characterized any further.Given ongoing concern for a lymphoproliferative process, she was referred for video-assisted thoracoscopic surgery (VATS). Pathology from her left upper and lower lobar wedge resections demonstrated follicular bronchiolitis. Biopsy of a pleural nodule demonstrated a lymphoproliferative process concerning for a low-grade B-cell neoplasm, thought to be extranodal PMZL. She was referred to oncology where PET/FDG demonstrated hypermetabolic multi-station adenopathy suggesting stage IV disease. Given her asymptomatic nature and anticipated indolent course, it was decided to defer chemotherapy pending resolution of the COVID-19 pandemic. DISCUSSION: MALT lymphoma can develop in response to continuous antigen stimulation, as is seen in Sjogren's syndrome or tobacco use. Radiographic features of MALT lymphoma are numerous, including consolidations, nodules, and masses being the most frequent patterns observed in ~50% of patients. Endobronchial lesions as observed in this patient occur in ~85% of cases. PET/FDG imaging commonly demonstrates mild uptake within identifiable lesions. It has a very favorable prognosis with an overall 5-year survival of 90% due to its indolent nature and excellent response rate to chemoimmunotherapy, typically combination Bendamustine and Rituximab. CONCLUSIONS: MALT lymphoma is an uncommon neoplasm that can occur due to chronic antigen stimulation, especially in patients with Sjogren's syndrome. It has numerous radiographic features, the most common being endobronchial lesions. When given these clinical features, establishing a diagnosis is of utmost importance due to the excellent response rate to therapy and favorable long-term prognosis. 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: Husnain M, Kuker R, Reis IM, et al. Clinical and radiological characteristics of patients with pulmonary marginal zone lymphoma: A single center analysis. Cancer Med. 2020;9(14):5051-5064. REFERENCE #3: Stefanovic A, Morgensztern D, Fong T, Lossos IS. Pulmonary marginal zone lymphoma: a single centre experience and review of the SEER database. Leuk Lymphoma. 2008;49(7):1311-1320. DISCLOSURES: No relevant relationships by Brandon Jakubowski, source=Web Response No relevant relationships by Rosechelle Ruggiero, source=Web Response
(Read More)