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- W4387248951 abstract "SESSION TITLE: Lung Cancer Case Report Posters 9 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus-associated lymphoproliferative disorder. It is characterized by atypical B-cell infiltration and granulomatosis, and commonly involves the lungs (pulmonary LG or PLG) but can also involve the central nervous system (CNS), skin, kidney, and liver. Patients often present with nonspecific respiratory symptoms and chest radiograph demonstrating multiple pulmonary lesions. Here, we describe a case of PLG with a unique presentation characterized by progressive neurologic symptoms. CASE PRESENTATION: A 66-year-old male with autism, atrial fibrillation, and congestive heart failure presented to the emergency department with progressively worsening dysphagia and generalized weakness. Symptoms began 6 months prior to admission after a mild respiratory illness. The patient had initially presented to an outside hospital where chest x-ray had revealed multiple lung masses. Biopsy at that time was inconclusive. On exam, vitals were unremarkable. The patient had mild left facial numbness in V1/V2 distribution, dysphonia, and left dysmetria. Cardiopulmonary exam were normal. Initial lab work was unremarkable. Magnetic resonance imaging (MRI) of the cervical spine and brain demonstrated metastatic lesions to the cervical spinal cord and medulla. A positron emission tomography (PET) scan obtained demonstrated PET-avid multiple pulmonary nodules and a left adrenal mass. Hospital course was complicated by new recurrent episodes of respiratory distress, wherein repeat work-up demonstrated stable pulmonary nodules. Transthoracic lung biopsy was inconclusive. A transbronchial biopsy of the right-lower lobe revealed non-caseating granulomatous disease. Patient then underwent a limited thoracotomy with wedge resection. Biopsy results were consistent with PLG. DISCUSSION: PLG has been reported to initially present with respiratory symptoms concerning for pneumonia, not unsurprisingly given the degree of disease burden in the lungs. There are reports of primary CNS LG in the literature, but it is either solely affecting the CNS or as a late-stage complication of PLG. Here we have described a patient with PLG whose initial symptoms were primarily neurologic, related to CNS metastasis, including his recurrent respiratory distress, likely secondary to lesions in the respiratory centers of the medulla. Due to its rarity, many clinicians may not be familiar with PLG and may neglect it when building a broad differential, leading to significant morbidity and mortality. We emphasize the importance of timely diagnosis and the role of open lung biopsies in PLG. CONCLUSIONS: LG is a very rare hematologic malignancy, able to affect multiple organ systems, and masquerade as other disease processes. This case discusses the challenges of diagnosing LG in patients with unusual presentations and highlights the importance of obtaining open lung biopsies for confirmation. REFERENCE #1: Lundell, RB, Weenig, RH, Gibson, LE. Chapter 11: Lymphomatoid Granulomatosis In: Rosen, ST eds. Rare Hematologic Malignancies. 1st ed. Springer; 2008: 265-272. REFERENCE #2: Melani C, Jaffe ES, Wilson WH. Pathobiology and treatment of lymphomatoid granulomatosis, a rare EBV-driven disorder. Blood. 2020;135(16):1344-1352. doi:10.1182/blood.2019000933 REFERENCE #3: Xu B, Liu H, Wang B, et al. Fever, Dry Cough and Exertional Dyspnea: Pulmonary Lymphomatoid Granulomatosis Masquerading as Pneumonia, Granulomatosis with Polyangiitis and Infectious Mononucleosis. Intern Med. 2015;54(23):3045-3049. doi:10.2169/internalmedicine.54.4822 DISCLOSURES: No relevant relationships by Doris Chan No relevant relationships by Jennifer Grinceri No relevant relationships by Abdulhaadi Khan No relevant relationships by Jonathan Park No relevant relationships by Fady Youssef" @default.
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- W4387248951 date "2023-10-01" @default.
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- W4387248951 title "TISSUE IS THE ISSUE: DIAGNOSING PULMONARY LYMPHOMATOID GRANULOMATOSIS WITHOUT RESPIRATORY SYMPTOMS" @default.
- W4387248951 doi "https://doi.org/10.1016/j.chest.2023.07.2941" @default.
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