Primary central nervous system lymphoma (PCNSL) is a non-Hodgkin’s lymphoma confined to the craniospinal axis and/or eyes, without evidence of systemic spread. Almost all PCNSL’s are classified as large B-cell lymphoma. A rare variant of PCNSL includes lymphomas of the T-cell phenotype. Until the publication of a large retrospective series of patients (pts) (n = 45) with T-cell PCNSL (

Shenkier TN, et al. J Clin Oncol 2005;23:2233–2239
),only case reports of T-PCNSL and literature reviews were available. The large retrospective series reported that ECOG Performance Status (PS) (0 or 1 versus 2, 3 or 4) at diagnosis was significantly associated with better outcome (Log rank P <.0001), as was primary treatment with high-dose methotrexate (MTX) (yes versus no) (Log rank P = .002). Median survival in the large series was 25 months (95% CI: 11 to 38 months). We reviewed our brain tumor database for pts seen between January 1980 and July 2008. Of 159 pts diagnosed with PCNSL, 3 (2M/1F) had histopathologically confirmed large T-cell phenotype. At diagnosis, the pts’ ages were 10, 23, and 64 years; in each case ECOG PS at diagnosis was 0. Brain parenchyma disease location was periventricular (1 pt), parietal (1 pt), and corpus callosum (1 pt); one pt had both brain and ocular lymphoma. First-line treatment was IA MTX-based chemotherapy with osmotic blood-brain barrier disruption (BBBD), without brain irradiation. The dose of MTX was 2.5 grams/day for 2 consecutive days, every 4 weeks for up to one year. A comprehensive neuropsychological test battery was administered at baseline prior to MTX-based BBBD treatment. Followup neuropsychological testing was conducted within 6 months after the final BBBD treatment. All patients attained CR and are alive 15.8, 8.9 and 1.2 years after diagnosis, with excellent PS and no evidence of disease progression to date. The 3 pts showed stable or improved neuropsychological function in all cognitive domains at follow-up testing. Side effects during BBBD treatment included focal seizures (1 pt) and atrial fibrillation (1 pt). In these 3 cases of T-cell PCNSL, encouraging tumor responses and survival were seen, with stable or improved neurocognitive functioning to date, warranting further investigation of CNS directed therapy in this rare variant of non-Hodgkin’s lymphoma.

Topics:
lymphoma, non-hodgkin, phenotype, primary central nervous system lymphoma, t-lymphocytes, blood-brain barrier disruption, brachial plexus neuritis, electrocorticogram, lymphoma, neoplasms, adverse effects

Disclosures: No relevant conflicts of interest to declare.

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2008, The American Society of Hematology
2008
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