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Primary CNS Lymphoma of T-Cell Origin: A Descriptive Analysis From the International Primary CNS Lymphoma Collaborative Group

From the Division of Medical Oncology, British Columbia Cancer Agency, Vancouver; Department of Radiation Oncology, Princess Margaret Hospital, Toronto, Canada; Unite Cytokines et Cancers, Hôpital Edouard Herriot, Centre Leon Berard, Lyon, France; Neuro-Oncology Program, Mayo Clinic Cancer Center, Rochester, MN; Dr. Bernard Verbeeten Instituut, Tilburg, the Netherlands; Charite Campus Benjamin Franklin, Berlin, Germany; Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY; Department of Neurology and Neurosurgery, Oregon Health & Science University, Portland, OR; Department of Neurology and Pathology, Harvard Medical School; Massachusetts General Hospital, Boston, MA; Department of Radiochemotherapy and Pathology, San Raffaele H Scientific Institute, Milan, Italy; Department of Radiation Oncology, Newcastle Mater Hospital, Newcastle, Australia; and University of California San Francisco, San Francisco, CA

Address reprint requests to Tamara N. Shenkier, MD, British Columbia Cancer Agency, 600 W 10th Ave, Vancouver, BC, V5Z 4E6, Canada; e-mail: tshenkier{at}bccancer.bc.ca

PURPOSE: To describe the demographic and tumor related characteristics and outcomes for patients with primary T-cell CNS lymphoma (TPCNSL).

PATIENTS AND METHODS: A retrospective series of patients with TPCNSL was compiled from twelve cancer centers in seven countries.

RESULTS: We identified 45 patients with a median age of 60 years (range, 3 to 84 years). Twenty (44%) had Eastern Cooperative Oncology Group (ECOG) performance status (PS) of 0 or 1. Twenty-six (58%) had involvement of a cerebral hemisphere and sixteen (36%) had lesions of deeper sites in the brain. Serum lactate dehydrogenase was elevated in 7 (32%) of 22 patients, and CSF protein was elevated in 19 of 24 patients (79%) with available data. The median disease-specific survival (DSS) was 25 months (95% CI, 11 to 38 months). The 2- and 5-year DSS were 51% (95% CI, 35% to 66%) and 17% (95% CI, 6% to 34%), respectively. Univariate and multivariate analyses were conducted for age ( 60 v > 60 years), PS (0 or 1 v 2, 3, or 4), involvement of deep structures of the CNS (no v yes), and methotrexate (MTX) use in the primary treatment (yes v no). Only PS and MTX use were significantly associated with better outcome with hazard ratios of 0.2 (95% CI, 0.1 to 0.4) and 0.4 (95% CI, 0.2 to 0.8), respectively.

CONCLUSION: This is the largest series ever assembled of TPCNSL. The presentation and outcome appear similar to that of B cell PCNSL. PS 0 or 1 and administration of MTX are associated with better survival.

Authors’ disclosures of potential conflicts of interest are found at the end of this article.

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