A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

Mohyuddin, Ghulam Rehman; Sultan, Fatima; Khaleeq, Ghulam

doi:10.1155/2012/371490

Cited by 3 publications

(8 citation statements)

References 12 publications

(14 reference statements)

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“…The lesions can progress rapidly, coalesce and commonly cavitate, therefore mimicking Wegner's granulomatosis or metastases 6 . In rare cases, it can present as a solitary large pleural based mass, idiopathic interstitial pneumonia or a lung abscess 6 , 7 . In our case, along with multiple lung nodules, there was also an endobronchial mass causing atelectasis, which is a rare presentation.…”

Section: Discussionmentioning

confidence: 99%

“…In our case, along with multiple lung nodules, there was also an endobronchial mass causing atelectasis, which is a rare presentation. There have only been two reports that describe of endobronchial involvement in previous literature 7 . However, our case is the only case to show complete regression of endobronchial mass and atelectasis after therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Twenty percent of stage 1 patients can achieve spontaneous remission, but the disorder is often progressive 10 . Studies have shown a median survival of 14 months and a mortality of 65%-90% 7 . Many patients die as a result of pulmonary hemorrhage, central nervous system involvement, or transformation to an aggressive lymphoma.…”

Section: Discussionmentioning

confidence: 99%

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Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass

et al. 2015

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Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive neoplastic proliferation of B and T lymphocytes commonly involving the lungs. Epstein-Barr virus is commonly detected in lesional cells. We report a case of a 54-year-old female with underlying monoclonal gammopathy of unknown significance who presented with a 4 week history of dyspnea and cough. Computed tomography scan of the chest showed multiple lung nodules as well as endobronchial narrowing causing atelectasis at the left upper lobe. Bronchoscopic findings revealed obstruction at the lingula segment due to endobronchial mass as a rare presentation. Bronchoscopic biopsy was diagnosed with LYG grade 1. After treatment, the endobronchial mass and lung lesions were completely resolved. However, the patient eventually evolved to malignant lymphoma after 1 year.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass

et al. 2015

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“…Lymphomatoid granulomatosis (LG), a rare Epstein Barr virus (EBV) associated B cell lymphoproliferative disorder, typically causes bilateral lower lobe lung nodules, and only rarely causes central airway obstruction [ 2 ]. Very few cases of LG endobronchial involvement have been reported in the English literature, two treated with chemotherapy [ 3 , 4 ] and one with pneumonectomy [ 5 ]. There is no data on bronchoscopic interventional options for these patients.…”

Section: Introductionmentioning

confidence: 99%

Central airway obstruction from lymphomatoid granulomatosis treated with an endobronchial stent

Greenberg

Jaitovich

Madisi

2022

Respiratory Medicine Case Reports

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“…Rarely, LYG presents as a solitary mass in the lung [8][9][10] . We report a rare radiographic presentation of pulmonary lymphomatoid granulomatosis as a single mass with the obstruction of right main stem bronchus.…”

Section: Introductionmentioning

confidence: 99%

Lymphomatoid Granulomatosis: Rare Radiologic Presentation as a Solitary Pulmonary Mass

Shestakova

Gallegos

Wang

et al. 2019

IJCO

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Background: Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. LYG is characterized by a progressive clinical course, which virtually always involves the lungs. LYG characteristically presents as bilateral pulmonary nodules. Pathologically, it is characterized by an angiocentric and angiodestructive infiltration of atypical EBV-positive B-lymphocytes admixed with reactive T-lymphocytes. We report a case of pulmonary LYG that presented as a large mass with complete occlusion of the right main stem bronchus intermedius in an 81-year-old female. Case: An 81-year-old female presented with shortness of breath to the emergency department. Inpatient imaging revealed bulky mediastinal lymphadenopathy with a right lower lobe collapse, shift of the cardiomediastinal silhouette, and a large right upper lung mass. Endobronchial ultrasound-guided biopsy (EBUS) revealed complete occlusion of right mainstem bronchus due to the right upper lung mass growth into the bronchus intermedius. Histopathological examination demonstrated clusters of large atypical EBV-positive B cells interspersed in a minimally polymorphous lymphocytic background, consistent with lymphomatoid granulomatosis, grade 3/3. Patient was treated with immunochemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone - CHOP) and Rituximab (anti-CD20 antibody). At the patient’s most recent follow-up, 6 months later, she was in a stable condition and her respiratory symptoms have improved. Conclusion: Lymphomatoid granulomatosis is a rare disease that should be considered in the differential diagnosis of a radiographic evaluation of a solitary pulmonary lung mass. Since the radiographic impression might favor carcinoma as the top differential diagnosis, biopsy of the lesion is paramount to ensure the correct diagnosis. Lymphomatoid granulomatosis is usually treated with an immunochemotherapy regimen with CHOP, and/or interferon, and Rituximab.

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A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

Cited by 3 publications

References 12 publications

Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass

Low Grade Pulmonary Lymphomatoid Granulomatosis with an Endobronchial Mass

Central airway obstruction from lymphomatoid granulomatosis treated with an endobronchial stent

Lymphomatoid Granulomatosis: Rare Radiologic Presentation as a Solitary Pulmonary Mass

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