Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

Masahiro Nagano; Takashi Ayaki; Natsuko Koita; Toshiyuki Kitano; Momoko Nishikori; Naoki Goda; Sachiko Minamiguchi; Akio Ikeda; Akifumi Takaori-Kondo; Ryosuke Takahashi

doi:10.2169/internalmedicine.1246-18

CASE REPORTS

Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

Masahiro Nagano, Takashi Ayaki, Natsuko Koita, Toshiyuki Kitano, Momoko Nishikori, Naoki Goda, Sachiko Minamiguchi, Akio Ikeda, Akifumi Takaori-Kondo, Ryosuke Takahashi

Author information

Masahiro Nagano
Department of Neurology, Kyoto University Graduate School of Medicine, Japan
Takashi Ayaki
Department of Neurology, Kyoto University Graduate School of Medicine, Japan
Natsuko Koita
Department of Neurology, Kyoto University Graduate School of Medicine, Japan
Toshiyuki Kitano
Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
Momoko Nishikori
Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
Naoki Goda
Department of Diagnostic Pathology, Kyoto University Graduate School of Medicine, Japan
Sachiko Minamiguchi
Department of Diagnostic Pathology, Kyoto University Graduate School of Medicine, Japan
Akio Ikeda
Department of Neurology, Kyoto University Graduate School of Medicine, Japan
Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine, Japan
Akifumi Takaori-Kondo
Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
Ryosuke Takahashi
Department of Neurology, Kyoto University Graduate School of Medicine, Japan

Keywords: primary central nervous system lymphoma (PCNSL), chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), recurrence, repeat brain biopsy, Epstein-Barr virus (EBV)

JOURNAL OPEN ACCESS

2019 Volume 58 Issue 6 Pages 849-854

DOI https://doi.org/10.2169/internalmedicine.1246-18

Details

Abstract

Primary central nervous system lymphoma (PCNSL) and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) can share clinical features and may be indistinguishable, even after brain biopsy. We encountered a case of Epstein-Barr virus-positive (EBV+) PCNSL recurrence in a patient with clinical features of CLIPPERS, and repeat brain biopsy was required to reach the correct diagnosis. Four years after the initial diagnosis and treatment of PCNSL, "peppering" punctate enhanced lesions with transient steroid responsiveness were detected during brain magnetic resonance imaging (MRI). A second brain biopsy supported a diagnosis of CLIPPERS, while a third biopsy confirmed the diagnosis of recurrent PCNSL.

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