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A Case of Rosai-Dorfman Disease Manifesting as Int ...
A Case of Rosai-Dorfman Disease Manifesting as Intracranial Dural-Based Tumors
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A 64-year-old man with coronary artery disease, hypertension, and recent 20-pound weight loss presented after a 10-minute seizure-like episode with transient altered mental status. Initial labs did not reveal an infectious source. Brain CT and MRI showed two dural-based extra-axial masses with vasogenic edema and mass effect, raising concern for meningioma or other dural lesions. CT scans of the chest, abdomen, and pelvis did not identify a primary malignancy. He was started on levetiracetam and dexamethasone, and EEG was negative for seizures.<br /><br />He later underwent elective left craniotomy with resection of the masses. Postoperative imaging showed decreased edema, reduced midline shift, and smaller residual extra-axial lesions. Pathology demonstrated chronic lymphohistiocytic and plasmacytic inflammation involving the meninges and dura with extension into the brain parenchyma, along with fibroconnective tissue proliferation. Immunohistochemistry was positive for S100, CD3, CD20, CD31, CD45, and CD68, and negative for CD1a and desmin, supporting Rosai-Dorfman disease (RDD). Histology also showed rare acid-fast bacilli, leading to concern for concomitant tuberculosis despite negative cultures and CSF studies.<br /><br />Because of the possible AFB findings, he was started on standard anti-tuberculous therapy with isoniazid, rifampin, ethambutol, pyrazinamide, and vitamin B6. Follow-up MRI later showed resolution of the lesions.<br /><br />This case highlights that extranodal RDD is a rare non-Langerhans cell histiocytic disorder that can present as isolated intracranial dural-based tumors and clinically mimic meningioma or tuberculoma. Surgical resection is the most effective treatment, and RDD should be considered in the differential diagnosis of dural masses.
Asset Subtitle
Amy Pulikeyil
Meta Tag
Author List
Amy Pulikeyil, Cristine K. Arcilla, Irina Mikolaenko, Natalia Plotskaya
Category
Clinical Vignettes
Concept
Rosai-Dorfman Disease
Concept
Surgical Resection
Concept
Intracranial Mass
Concept
Dural-Based Lesion
Concept
Histopathology
Distinguished
Non-Finalist
Presenter Organization
Capital Health Regional Medical Center, Trenton, NJ
Presenting Author
Amy Pulikeyil
Track
Adult
Keywords
Rosai-Dorfman disease
dural-based masses
intracranial lesion
meningioma mimic
seizure-like episode
vasogenic edema
craniotomy resection
histiocytic disorder
tuberculosis
extra-axial tumor
Rosai-Dorfman Disease
Surgical Resection
Intracranial Mass
Dural-Based Lesion
Histopathology
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