Lymphomatoid Granulomatosis: Rare Radiologic Presentation as a Solitary Pulmonary Mass

Abstract

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.