Clinical Characteristics of Primary T-cell Lymphoma of the Central Nervous System
Hannah Padilla1, Kim Griffin2, Derek Johnson2, Reece Hass3, Tony Zhang2, Ivan Carabenciov2, Rafid Mustafa4, Michel Toledano2
1Mayo Clinic Alix School of Medicine, 2Mayo Clinic, 3Mayo Clinic, Rochester, 4Mayo Clinic, Department of Neurology
Objective:
To characterize clinical presentation, imaging, and laboratory characteristics of patients with primary central nervous system (CNS) T-cell lymphoma (TCL).
Background:
Primary CNS TCL is a rare subset of an uncommon condition, as most primary CNS lymphomas are B-cell in origin. The rarity of literature creates diagnostic dilemmas in patients presenting with undifferentiated brain lesions, often delaying diagnosis.
Design/Methods:
This is a descriptive study of primary CNS TCL cases evaluated within Mayo Clinic between 1/1/2005 and 6/30/2023, highlighting demographics, clinical presentation, neuroimaging, and laboratory features. Pathologically confirmed diagnosis was required for inclusion.
Results:
Thirteen patients with primary CNS TCL were identified. Ten (77%) were male with a median age of 43 (IQR 32 – 52) at initial presentation. Presenting symptoms included seizure (5), focal neurologic deficit (4), headache (3), and cognitive impairment (1). Ten (77%) developed seizures during their course. All patients had enhancing lesions and supratentorial involvement, with corpus callosum infiltration in 5 (38%). Nine (69%) had blooming artifact on susceptibility weighted imaging. Eight (62%) had multiple lesions at presentation.
Eleven patients had cerebrospinal fluid testing, with 1 having cytology demonstrating nonspecific non-hematolymphoid malignant cells. Prior to biopsy confirmation, 6 patients received alternative diagnoses: chronic Lyme, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), CNS vasculitis, demyelinating disease, and encephalitis. All patients were diagnosed via brain biopsy, with 4 (31%) requiring two biopsies for diagnosis. Those requiring a second biopsy received an incorrect diagnosis following initial biopsy. Median time from presentation to diagnosis was 63 days (IQR 24 – 180).
Conclusions:
Primary CNS TCL should be considered for patients with enhancing brain lesions. A high index of suspicion is necessary, and over 30% of our patients required repeat brain biopsy for diagnosis. Diagnostic clues include supratentorial location, multiple lesions, callosal involvement, and intralesional blooming artifact on susceptibility weighted imaging.
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