Abstract Details

Title Peripheral T-Cell Lymphoma Manifesting as Multifocal Central Nervous System Disease with Prominent Spinal Cord Involvement

Topic Neuro-oncology

Presentation(s) P2 - Poster Session 2 (11:45 AM-12:45 PM)

Poster/Presentation
Number 11-001

Background Metastatic CNS neoplasms, although rare, should be considered early in rapidly declining patients with multifocal disease in whom immune-mediated or infectious etiologies have been excluded.

Objective To emphasize the need for timely and wide-reaching diagnostic testing in the evaluation of acute-onset multifocal CNS disease with prominent spinal cord involvement.

Design/Methods N/A

Results

A 53-year-old male with Celiac disease on azathioprine presented with one-week of vertigo, diplopia, and dysarthria upon one-year of malaise and cachexia. The patient’s examination revealed right eyelid ptosis, bilateral abducens palsies, restricted up-gaze, dysarthria, and diffuse dysmetria and hyporeflexia. MRI showed a right T2 hyperintense contrast-enhancing cerebellar lesion and a small left cerebellar diffusion-restricting lesion. CSF was inflammatory (nucleated cell 6 cells/mcL, protein 181 mg/dL, glucose 30 mg/dL). Workup for autoimmune encephalitis, myelin-oligodendrocyte-glycoprotein-IgG, aquaporin-4-IgG, and infections etiologies were negative. He was empirically treated with intravenous methylprednisolone and immunoglobulin without benefit.

He continued to decline and repeat CSF was again inflammatory (28 cells/mcL, protein 494 mg/dL), without clonal cells. Repeated MRI showed new left frontal leptomeningeal and cauda equina enhancement extending into the adjacent musculature. Electromyography showed proximal myopathy and subsequent biopsy was unrevealing. A bone marrow and brain biopsy were declined due to development of pulmonary embolism and multiorgan failure. He continued to decline and developed multifocal strokes, expiring 6 weeks after presentation. Autopsy demonstrated peripheral T cell lymphoma (PTLC).

Conclusions Intramedullary non-CNS spinal metastases represent less than 8.5% of all metastatic tumors and 3% of all spinal metastases. PTCL represents 15% of non-Hodgkin lymphomas and can rarely involve the CNS parenchyma or leptomeninges. Persistent enhancement, elevated protein, and minimal response to steroids or subsequent rebound should raise suspicion for neoplasm, especially considering these patients’ rapid decline can make CNS biopsy inadvisable later in the disease course.

Authors/Disclosures
Eesha Singh, MD (Vanderbilt University Medical Center) PRESENTER	Dr. Singh has nothing to disclose.
Richard Carozza, MD (Vanderbilt Children's Hospital)	Dr. Carozza has nothing to disclose.
Karl E. Misulis, MD, PhD, FAAN (Neurology Department, Vanderbilt University Medical Center)	Dr. Misulis has received publishing royalties from a publication relating to health care.
Shailee S. Shah, MD (Vanderbilt Multiple Sclerosis Center)	Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Shah has received publishing royalties from a publication relating to health care.