Objective:

To report the clinical spectrum and outcomes of patients with peripheral T-cell lymphoma (PTCL) who develop central nervous system (CNS) involvement.

Background:

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.

Design/Methods:

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).

Results:

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.  

Conclusions: