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Abstract Details

Central Nervous System Manifestations of Peripheral T-cell Lymphoma
Neuro-oncology
P4 - Poster Session 4 (8:00 AM-9:00 AM)
6-002
To report the clinical spectrum and outcomes of patients with peripheral T-cell lymphoma (PTCL) who develop central nervous system (CNS) involvement.
CNS involvement in PTCL is rare but often devastating, with heterogeneous presentations. CNS involvement may occur at initial diagnosis or as a relapse. Better characterization of its manifestations may aid early recognition and management.
Retrospective review of Mayo Clinic records between January 2005 and June 2023 for patients ≥18 years with pathologically confirmed systemic PTCL and coexisting CNS involvement, defined by neurological symptoms and/or supportive adjunctive testing (radiologic, laboratory, pathologic).
Forty-six patients met inclusion criteria. PTCL-not otherwise specified, was the most common subtype (43%). Median age was 59 years (IQR 49–67), with male predominance ( 76%). CNS disease was present at diagnosis in 54%. Presentations included cognitive change (43%), cranial neuropathies (30%), focal deficits (26%), and seizures (9%); 4/46 (9%) were asymptomatic but had supportive adjunctive testing. CNS biopsy was attempted in 10/46 and diagnostic in 9/10.  Distribution was leptomeningeal in 56%, parenchymal in 33%, and mixed in 11%. Cerebrospinal fluid analysis (n=39) demonstrated elevated protein in 87%, pleocytosis in 74%, and malignant cytology/flow cytometry in 72% . MRI abnormalities were detected in 36/44 (82%): 72% brain/brainstem lesions, 8% spine-only disease, and 19% both. Extranodal systemic disease was frequent, most often involving soft tissue (61%), skin (59%), and the gastrointestinal tract (46%). Mortality data were available for 42/46 (91%). Thirty-six of 42 patients (86%) died despite optimal chemotherapy.  Among 6/42 survivors (14%), 4/6 remained in remission, including 3/4 who received stem cell transplantation. Median Overall Survival (OS) was 13 months. OS at 1, 3, and 5 years was 54.6%, 27.4%, and 16.5%, respectively.  

CNS involvement in PTCL is clinically and radiographically diverse but associated with poor survival. These findings emphasize the need for improved strategies in risk stratification and treatment.

Authors/Disclosures
Rafid Mustafa, MD (Mayo Clinic, Department of Neurology)
PRESENTER
Dr. Mustafa has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon Therapeutics. Dr. Mustafa has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for PicnicHealth. Dr. Mustafa has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Annexon Biosciences. Dr. Mustafa has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Annexon Biosciences.
Tony Zhang, MBChB Dr. Zhang has nothing to disclose.
Reece Hass, DO (Mayo Clinic, Rochester) Dr. Hass has nothing to disclose.
Hannah Padilla Miss Padilla has nothing to disclose.
Kim Griffin, MD (Mayo Clinic) Dr. Griffin has nothing to disclose.
Derek W. Stitt, MD (Mayo Clinic) Dr. Stitt has nothing to disclose.
Ivan D. Carabenciov, MD (Mayo Clinic) Dr. Carabenciov has nothing to disclose.
Michel Toledano, MD (Mayo Clinic) Dr. Toledano has nothing to disclose.