title: Primary central nervous system gamma delta cytotoxic T-cell lymphoma.
abstract: Primary T-cell lymphomas of the central nervous system (CNS) are uncommon, but aggressive and increasing in incidence. We describe a rare case of T-cell lymphoma in a cerebellar location, to our knowledge the first reported case demonstrating gamma/delta receptor expression. Additionally, we elaborate on key diagnostic features and review all nine patients with primary CNS lymphoma of cytotoxic T-cell phenotype reported in the literature. A 26-year-old female medical student presented with a 6 week history of nausea, vomiting and dizziness. MRI revealed a 2 cm cerebellar mass. The tumor was subtotally resected, and pathologic examination of a subtotal resection specimen demonstrated peripheral T-cell lymphoma, not otherwise specified, with a gamma/delta cytotoxic T-cell phenotype. She subsequently started high dose methotrexate and cytarabine. We report a unique case of primary CNS gamma delta CD8+ T-cell lymphoma lineage in a young female patient. While these are rare entities, it is an important differential diagnosis to consider. Therapy should be tailored to the patient, and involves resection with adjuvant chemotherapy, radiotherapy or autologous stem-cell based treatments.
authors.affiliation: Department of Pathology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Kelly L
authors.initials: KL
authors.lastname: Mooney
authors.affiliation: Department of Neurosurgery, David Geffen School of Medicine at University of California, Los Angeles, 650 Charles E. Young Drive, Wasserman Building, Room 562, CA 90095-1761, USA.
authors.forename: Winward
authors.initials: W
authors.lastname: Choy
authors.affiliation: Department of Neurosurgery, David Geffen School of Medicine at University of California, Los Angeles, 650 Charles E. Young Drive, Wasserman Building, Room 562, CA 90095-1761, USA.
authors.forename: Joslyn
authors.initials: J
authors.lastname: Woodard
authors.affiliation: Department of Pathology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Rena R
authors.initials: RR
authors.lastname: Xian
authors.affiliation: Department of Pathology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Taylor M
authors.initials: TM
authors.lastname: Deal
authors.affiliation: Department of Hematology and Oncology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Ryan F
authors.initials: RF
authors.lastname: Kendle
authors.affiliation: Department of Pathology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.affiliation: Department of Pathology, David Geffen School of Medicine at University of California, Los Angeles, CA, USA.
authors.forename: Wayne
authors.initials: W
authors.lastname: Grody
authors.affiliation: Department of Neurosurgery, David Geffen School of Medicine at University of California, Los Angeles, 650 Charles E. Young Drive, Wasserman Building, Room 562, CA 90095-1761, USA. Electronic address: iyang@mednet.ucla.edu.
authors.forename: Isaac
authors.initials: I
authors.lastname: Yang
mesh_terms: D000328:Adult; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D006801:Humans; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013601:T-Lymphocytes / Q000473:pathology; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology*
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta
keywords: Brain; CNS; Cytotoxic; Gamma/delta; Lymphoma; T-cell