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Not Acing It: Primary CNS Lymphoma in Setting of ...
Not Acing It: Primary CNS Lymphoma in Setting of Neurosarcoidosis
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This case describes a 58-year-old woman with known sarcoidosis and neurosarcoidosis who developed worsening neurologic deficits, fever, and multiple enlarging ring-enhancing brain lesions. Because neurosarcoidosis can mimic many other conditions, her lesions were initially treated as possible brain abscesses versus progression of neurosarcoidosis. She was discharged on broad-spectrum antibiotics, but returned weeks later with new left-sided weakness and persistent systemic illness. <br /><br />Imaging showed increasing size and number of peripherally enhancing lesions with marked vasogenic edema and midline shift. Her CSF was inflammatory but nonspecific, and infectious studies were negative. Despite antibiotics and dexamethasone, her condition worsened. A delayed brain biopsy, performed after steroid exposure, showed an atypical extensively necrotic lesion with mixed B- and T-cell infiltrates adjacent to necrotic CD20-positive cells, raising strong concern for primary CNS lymphoma. Additional cultures remained negative, and PET imaging showed disease confined to the brain without systemic involvement. <br /><br />She was treated with R-MPV chemotherapy and zanubrutinib, but there was no clinical or radiographic improvement after several months. She ultimately transitioned to comfort care. <br /><br />The case highlights several important teaching points: <br />- Ring-enhancing brain lesions have a broad differential, especially in immunocompromised patients. <br />- Neurosarcoidosis can obscure the diagnosis of CNS lymphoma. <br />- Peripheral diffusion restriction and lesion enlargement despite antibiotics should prompt concern for malignancy. <br />- High-dose steroids and delayed biopsy can reduce diagnostic yield. <br /><br />The authors emphasize that primary CNS lymphoma is rare but should remain on the differential in patients with sarcoidosis and progressive neurologic disease, and that early brain biopsy is crucial when the diagnosis is uncertain.
Asset Subtitle
Rose Nganga
Meta Tag
Author List
Ann Marie Kumfer, Rose Nganga
Category
Clinical Vignettes
Concept
Neurosarcoidosis
Concept
Primary Central Nervous System Lymphoma
Concept
Brain biopsy
Concept
Differential Diagnosis
Concept
Cerebral Abscess
Distinguished
Non-Finalist
Presenter Organization
UNC Hospital
Presenting Author
Rose Nganga
Track
Adult
Keywords
sarcoidosis
neurosarcoidosis
primary CNS lymphoma
ring-enhancing brain lesions
brain biopsy
vasogenic edema
diffusion restriction
CD20-positive cells
R-MPV chemotherapy
zanubrutinib
Neurosarcoidosis
Primary Central Nervous System Lymphoma
Brain biopsy
Differential Diagnosis
Cerebral Abscess
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