Primary central nervous system cytotoxic/suppressor T-cell lymphoma: report of a unique case and review of the literature

• Published in: The American journal of surgical pathology Vol. 27; no. 5; p. 682
• Main Authors: Liu, Dating, Schelper, Robert L, Carter, David A, Poiesz, Bernard J, Shrimpton, Anthony E, Frankel, Bruce M, Hutchison, Robert E
• Format: Journal Article
• Language: English
• Published: United States 01.05.2003
• Subjects: Aged; Aged, 80 and over; Antigens, CD - analysis; Biomarkers, Tumor - analysis; Brain - pathology; Brain Neoplasms - chemistry; Brain Neoplasms - genetics; Brain Neoplasms - pathology; DNA, Neoplasm - analysis; Fatal Outcome; Female; Gene Rearrangement, T-Lymphocyte - genetics; Genes, T-Cell Receptor - genetics; Humans; Immunohistochemistry; Lymphoma, T-Cell, Peripheral - chemistry; Lymphoma, T-Cell, Peripheral - genetics; Lymphoma, T-Cell, Peripheral - pathology; Magnetic Resonance Imaging; Polymerase Chain Reaction; T-Lymphocyte Subsets - chemistry; T-Lymphocyte Subsets - pathology; T-Lymphocytes, Cytotoxic - chemistry; T-Lymphocytes, Cytotoxic - pathology; T-Lymphocytes, Regulatory - chemistry; T-Lymphocytes, Regulatory - pathology; Tomography, X-Ray Computed
• ISSN: 0147-5185
• DOI: 10.1097/00000478-200305000-00012

Peripheral T-cell lymphoma primary to the central nervous system is a rare occurrence. The authors report a case of an 89-year-old woman who presented with a 3-month history of worsening confusion and recent onset of headache, nausea and vomiting, and upper limb tremors. Computed tomography and magnetic resonance imaging examinations demonstrated a 4.5-cm solitary brain mass in the right basal ganglia with compression along the ventricular system. No other lesion was found in the patient. Histologic and immunohistochemical studies of a stereotactic biopsy of the mass showed a T-cell lymphoproliferative lesion positive for CD3, CD8, CD57, and T-cell intracellular antigen 1 and negative for CD4, CD56, CD30, anaplastic lymphoma kinase, and CD20. A monoclonal T-cell receptor-gamma gene rearrangement was detected by polymerase chain reaction analysis of genomic DNA isolated from paraffin-embedded tumor tissue sections. These findings were consistent with peripheral T-cell lymphoma of cytotoxic/suppressor phenotype, resembling the phenotype of T-cell large granular cell leukemia. To the authors' best knowledge, this represents the first reported case of primary brain T-cell lymphoma with a cytotoxic/suppressor immunophenotype. A brief review of the literature of primary brain T-cell lymphoma is also presented.