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Abstract Details

Primary Central Nervous System T-cell Lymphoma Mimicking Isolated CNS Angiitis in a Young Immunocompetent Woman
Neuro-oncology
P1 - Poster Session 1 (9:00 AM-5:00 PM)
422

To present a challenging case initially diagnosed as primary angiitis of the central nervous system (CNS).

Primary CNS T-cell lymphoma is a rare CNS neoplasm that frequently mimics other conditions.

Case report 
We report the case of 17-year-old woman presenting with cognitive deficits that gradually worsened over two months. A brain MRI revealed multiple regions of high T2 signal with bilateral involvement of the fronto-temporo-insular areas, basal ganglia and left thalamus. Brain MRA and whole-body PET scan were normal. An exhaustive work-up for autoimmune, rheumatological, genetic and infectious conditions was unremarkable. The patient received a course of intravenous methylprednisolone and presented three months later with worsening headaches associated with somnolence. A repeat brain MRI showed multiple new bilateral cortical-subcortical enhancing lesions with surrounding edema in the right peri-insular area and left cingulate gyrus. CSF studies including cytology were unremarkable. A brain biopsy showed aggressive angiocentric chronic inflammation with a parenchymal infiltrate predominantly composed of T lymphocytes- results consistent with a vasculitis or primary angiitis of the CNS although other conditions such as infection, immune mediated process or lymphoma could not be excluded. T-cell receptor gene rearrangement (TCR) revealed a clonal population of TCR-gamma, diagnostic of a primary CNS T-cell lymphoma, not otherwise specified, CD30 negative with a cytotoxic phenotype. A bone marrow biopsy showed hypocellular marrow without abnormal infiltrate. Treatment with methotrexate, procarbazine and vincristine was initiated.  However, the patient’s condition gradually deteriorated, and she passed away two months after initiation of therapy following a septic shock. 
Primary CNS T-cell lymphoma is a very rare condition especially in young adults. It is difficult to distinguish it from other conditions clinically, radiologically and histopathologically. Diagnosis requires a high level of suspicion and TCR should be performed when atypical features are present.
Authors/Disclosures
Badiee A. Moussa, MD (American University of Beirut Medical Center)
PRESENTER 		Dr. Moussa has nothing to disclose.
Fatima Mallat, MD (Hamad Medical Corporation) Dr. Mallat has nothing to disclose.
Georgette Dib, MD Dr. Dib has nothing to disclose.
No disclosure on file
No disclosure on file