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Abstract Details

Lymphomatoid Granulomatosis: A Rare Lymphoproliferative Disorder on the Differential Diagnosis for Brain and Spinal Cord Lesions
Neuro-oncology
P4 - Poster Session 4 (11:45 AM-12:45 PM)
5-027
To convey the importance of considering lymphomatoid granulomatosis as a differential diagnosis in patients presenting with steroid-responsive pulmonary and neurologic lesions.  
Lymphomatoid granulomatosis is a rare EBV-driven angioinvasive and angiodestructive B-cell lymphoproliferative disorder. Lungs are most commonly affected, followed by the central nervous system (CNS), skin, kidneys, and liver. Within the CNS, spinal cord involvement is rare.
N/A

A 58-year-old woman with an incidental subpleural nodule found in 2020 during evaluation of exertional angina developed persistent respiratory symptoms after COVID-19 infection. PET CT demonstrated active lesions in the lungs and frontoparietal lobes. After initial diagnosis of squamous cell carcinoma (SCC) on endobronchial fine needle aspirates, a brain biopsy found perivascular and intraparenchymal inflammatory changes without evidence of malignancy. SCC was not confirmed in subsequent biopsies. Rheumatologic evaluation of diffuse arthralgias was unremarkable. Serial MRI brain revealed resolution of previously enhancing lesions after steroid treatment and new regions of parenchymal and leptomeningeal enhancement.  

Three months later, she developed left leg weakness and paresthesias. MRI revealed new contrast-enhancing cerebellar and occipital lesions, as well as a longitudinal partially ring-enhancing lesion in the thoracic spinal cord. Imaging also demonstrated  lesions in the lungs and lumbar paraspinal musculature. Spinal fluid studies showed 39 nucleated cells (lymphocyte predominant), 129 mg/dl protein, and normal glucose. Repeat lung biopsy with wedge resection confirmed grade III lymphomatoid granulomatosis. High-dose steroids provided near-resolution of symptoms. She is receiving systemically directed chemotherapy in a clinical trial with dramatic reduction in lung and CNS lesion burden. 

Lymphomatoid granulomatosis is a rare disease involving the lungs and brain most commonly, with rare involvement of the spinal cord. Prompt treatment is critical because this disease can progress to diffuse large B-cell lymphoma. This case demonstrates the importance of pursuing biopsy in the setting of steroid-responsive lesions in the brain, spinal cord, and lungs.
Authors/Disclosures
Sneha Lingam, MD (Atrium Health Wake Forest Baptist)
PRESENTER
Ms. Lingam has nothing to disclose.
Dhvani Raghupathy No disclosure on file
Keirsyn Criss No disclosure on file
Deepal P. Shah-Zamora, MD (Atrium Health Wake Forest Baptist) Dr. Shah-Zamora has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi Winthrop Industrie. The institution of Dr. Shah-Zamora has received research support from CereGate Inc. The institution of Dr. Shah-Zamora has received research support from Amneal. The institution of Dr. Shah-Zamora has received research support from AbbVie .
Emily P. Pharr, MD, FAAN Dr. Pharr has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.
Rakhee Vaidya (Atrium Health Wake Forest) No disclosure on file
Kathryn Mercer No disclosure on file
Danielle Maracaja (Wake Forest University School of Medicine) No disclosure on file
Roy E. Strowd III, MD, FAAN (Wake Forest School Of Medicine) Dr. Strowd has received personal compensation for serving as an employee of Kaplan. Dr. Strowd has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Monteris Medical, Inc. Dr. Strowd has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novocure. The institution of Dr. Strowd has received personal compensation in the range of $500-$4,999 for serving as a Consultant for SpringWorks . Dr. Strowd has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. The institution of Dr. Strowd has received research support from Southeastern Brain Tumor Foundation. The institution of Dr. Strowd has received research support from Jazz Pharmaceuticals. The institution of Dr. Strowd has received research support from National Institutes of Health. The institution of Dr. Strowd has received research support from Alpha Omega Alpha. The institution of Dr. Strowd has received research support from American Board of Psychiatry and Neurology. Dr. Strowd has received publishing royalties from a publication relating to health care. Dr. Strowd has received publishing royalties from a publication relating to health care.