What does this research mean for the field?

Pulmonary MALT lymphoma can develop atypically in patients with long-standing, dormant pulmonary sarcoidosis and should be considered when a new or non-resolving infiltrate appears. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

To present a rare case of pulmonary MALT lymphoma associated with long-standing pulmonary sarcoidosis.

May 20, 2026

C40-12 Air on the Side of Caution: A Typical Radiographic Presentation of Rare Pulmonary Lymphoma-associated With Sarcoidosis

Key Points

To present a rare case of pulmonary MALT lymphoma associated with long-standing pulmonary sarcoidosis.
Described a 76-year-old male with a history of pulmonary sarcoidosis diagnosed 17 years prior.
Conducted navigational bronchoscopy, transbronchial biopsy, and endobronchial ultrasound for diagnosis.
Used imaging (CT and PET/CT) to assess lymphadenopathy and lymphoproliferative disorder.
Diagnosis of pulmonary MALT lymphoma confirmed via biopsy and characteristic imaging.
Patient exhibited abnormal CXR and elevated IgA level of 1905 mg/dl prior to treatment.
Three months post-treatment with rituximab, PET/CT showed significant improvement.

Abstract

Abstract Introduction Pulmonary mucosal-associated lymphoid tissue (MALT) lymphoma is an exceedingly rare form of lymphoma. Pulmonary MALT lymphoma associated with pulmonary sarcoidosis (PS) is even more uncommon with only four documented case reports. We present a case of pulmonary MALT lymphoma in a patient with long-standing quiescent pulmonary sarcoidosis. Case Description A 76-year-old male was referred to pulmonary clinic for an abnormal chest x-ray (CXR). PS was diagnosed after a routine CXR discovered hilar lymphadenopathy 17 years earlier. He denied any symptoms of extrapulmonary sarcoidosis. His pulmonary function tests as well as serial CXRs were normal. He never required treatment. Three months prior to his presentation he developed shortness of breath and a cough productive of clear phlegm without fever. His CXR demonstrated an infiltrate in the right upper lobe (RUL) and stable chronic hilar lymphadenopathy. He was treated for pneumonia twice over the course of a month without any improvement. Two months after his initial presentation, an interval CXR was unchanged, so a CT chest was obtained (Figure 1). His physical exam was unremarkable. Due to the CT findings, he underwent navigational bronchoscopy with needle and transbronchial biopsy (TBBx) of the RUL followed by endobronchial ultrasound (EBUS) and biopsy of lymph node stations 4L and 7. Flow cytometry revealed a mature CD5 negative/CD10 negative lymphoproliferative disorder with lambda-restricted plasma cells. Surgical pathology from the TBBx was consistent with mature B-cell non-Hodgkin’s lymphoma (NHL). The EBUS was nondiagnostic and microbiologic studies were negative. A whole body PET/CT demonstrated hypermetabolic lymphadenopathy above and below the diaphragm with low level FDG-avidity of the right and left hilar regions. His IgA level was 1905 mg/dl (70-400 mg/dl). The patient was ultimately diagnosed with pulmonary MALT lymphoma based on the typical radiographic findings, the elevated IgA level, and the known association with chronic inflammatory disorders such as sarcoidosis. He was treated with rituximab monotherapy. Three months later, his follow up PET/CT scan demonstrated interval improvement. Discussion Sarcoidosis mimics many disease processes and one must err on the side of caution when there are changes. PS and MALT lymphoma are non-specific but air bronchograms might be the key to differentiation. Lymphoma may develop in the setting of sarcoidosis however usually occurring within 2 years after diagnosis as active disease. MALT lymphoma presenting in a patient with seemingly dormant sarcoidosis is atypical but must be considered when a patient with PS develops a new or non-resolving infiltrate. This abstract is funded by: None

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