Retrospective Analysis of 9 Cases of Primary Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma and Literature Review

Abstract

Background Primary pulmonary mucosa-associated lymphoma tissue lymphoma is rare and is often misdiagnosed because of its diverse and nonspecific clinical features. The aim of this study was to raise awareness among clinicians and to share our experience of treating and managing such patients. Material/Methods This retrospective study was conducted between 1 January 2009 and 31 October 2017 at the First Affiliated Hospital of Guangxi Medical University. All cases were confirmed via pathology and immunohistochemistry. In addition, we reviewed all relevant literature. Results Altogether, 21 patients (7 female, 14 male) with a median age of 54 (range, 19–84) years were diagnosed with primary pulmonary mucosa-associated lymphoma. Expiratory dyspnea, repeated cough and expectoration, and weight loss were the most common symptoms. Pulmonary lesions were found via physical examination in 10 patients who had no obvious symptoms. Chest computed tomography showed nodules, pulmonary consolidation, bronchial bronchogram, ground-glass opacity, and mediastinal lymph node enlargement. Some patients were misdiagnosed with tuberculosis and pneumonia, while others were initially diagnosed with cancer. Tumor pathology and immunocytochemistry indicated primary pulmonary mucosa-associated lymphoma tissue lymphoma. Six patients underwent chemotherapy, 5 underwent surgery, 4 underwent surgery and chemotherapy, 3 were only observed, and 3 refused treatment. Conclusions The development of primary pulmonary mucosa-associated lymphoid tissue lymphoma is slow and insidious. Having no specific clinical symptoms and imaging findings, it is easily misdiagnosed. Final diagnosis is made via pathologic evaluation and immunohistochemistry. Surgery and chemotherapy are the primary treatment modalities and yield a good prognosis.

Two rare cases of bronchus‐associated lymphoid tissue lymphoma successfully treated with rituximab‐bendamustine

“…If BALT lymphoma is localized and accessible, surgery is also a possible treatment option compared with chemotherapy. 17 , 18 , 25 Our patients showed bi‐pulmonary and extrapulmonary manifestations. Therefore, local surgery was not an option and an immunochemotherapy with rituximab and bendamustine was performed.…”

“…In line with previous reports, CT scan showed solitary pulmonary nodules in all lung lobes. 18 , 25 , 26 , 27 Based on the CT scan, a biopsy of lung tissue, clonality, and immunohistochemistry analysis were performed as recommended (NCI SEER database). 1 , 10 The clonality analysis detected immunoglobulin gene rearrangements in IGHA, IGHB, and IGHC, which have previously been described to be affected in BALT lymphomas.…”

“…In previous case reports and studies, rituximab as single agent or combined therapy with chlorambucil, cladribine, or CHOP led to good responses with long‐term survival up to 70%. 18 , 20 , 44 , 45 Finally, radiation and eradication therapies are also investigated (ClinicalTrials.gov: NCT01820910, NCT03680586, NCT02494700).…”

Maltoma veiled in the lung-a rare case of pulmonary and gastric maltoma

“…Chemotherapy with rituximab-chlorambucil is considered first-line as the combination has shown a 5-year survival rate in 68% of patients [ 18 ]. Other viable regimens include fludarabine and bortezomib [ 19 ]. Due to findings of a pancreatic malignancy in our patient, the chosen chemotherapy regimen was aimed at the pancreatic cancer, with simultaneous activity against pMALToma.…”

Incidence, prognostic factors and clinical practice of Primary Pulmonary Lymphoma: A large population-based retrospective study

“…Current knowledge about PPL was mainly originated from several retrospective studies conducted in the past few years, but the enrolled number of patients were range from nine to thirty (Du C et al 2018a, Cozzi et al 2019a). Individual case reports with small series along with retrospective studies mentioned above weren't able to deliver information concerning incidence, treatment modality, and survival.…”