Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma

Authors: Imai, Hiroshi; Shimada, Kazuyuki¹; Shimada, Satoko²; Abe, Masato³; Okamoto, Masataka⁴; Kitamura, Kunio⁵; Kinoshita, Tomohiro¹; Shiraishi, Taizo⁶; Nakamura, Sigeo²

Source: Pathology International, Volume 59, Number 7, July 2009 , pp. 431-437(7)

Publisher: Wiley-Blackwell

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Abstract:

Primary CNS diffuse large B-cell lymphoma (CNS DLBCL) is confined to the CNS, and constitutes a distinct entity. In the present study a series of 40 Japanese patients with CNS DLBCL who presented with neurological, but not systemic symptoms, was reviewed. Median survival was 18.7 months. CD5, CD10, Bcl-6, MUM-1, and Bcl-2 were positive in 30%, 10%, 84%, 100%, and 93% of patients, respectively. All CD10-negative patients had non-germinal center B-cell type. There was no significant difference in survival among the immunophenotypic subgroups. CNS DLBCL appeared to be homogenous as a group, which prompted the comparison with another distinct extranodal entity, intravascular large B-cell lymphoma (IVLBCL) in Japanese patients. CNS DLBCL patients did not differ in age, sex, or immunophenotype, including CD5 positivity, from IVLBCL patients, but were significantly less likely to have poor prognostic parameters than IVLBCL patients: the international prognostic index score was low or low-intermediate in 86% of CNS DLBCL patients and high or high-intermediate in 98% of IVLBCL patients. Notably, despite this difference, their survival curves almost overlapped. The present study highlights the issue of clinical distinctiveness of aggressive extranodal lymphomas, the peculiar migration and localization of which should be further clarified.

Keywords: CD5; intravascular large B-cell lymphoma; primary central nervous system diffuse large B-cel

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1440-1827.2009.02390.x

Affiliations: 1: Hematology and Oncology and 2: Pathology and Biological Response, Nagoya University Graduate School of Medicine, Nagoya, 3: Department of Pathology, Faculty of Medical Technology, Fujita Health University, School of Health Science, 4: Department of Internal Medicine, Division of Hematology and Medical Oncology, Fujita Health University, School of Medicine, Toyoake and 5: Department of Internal Medicine, Ichinomiya Municipal Hospital, Ichinomiya, Japan 6: Division of Pathologic Oncology, Mie University Graduate School of Medicine, Tsu, Departments of

Publication date: 2009-07-01

Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma

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