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A case of ‘lymphomatosis cerebri’ diagnosed in an early phase and treated by whole brain radiation: case report and literature review

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Abstract

‘Lymphomatosis cerebri’ (LC) is a term indicating a diffusely infiltrating form of primary central nervous system lymphoma (PCNSL) without evidence of a mass lesion. Not infrequently, diagnostic confusion is caused by its presentation on cranial magnetic resonance images (MRI), which is characterized by diffuse leukoencephalopathy without contrast enhancement. In this report, we describe a 53-year-old, immunocompetent man who had an insidiously progressive dementia and right weakness. On serial MRI in 4 months duration, diffuse white matter lesions without contrast enhancement gradually progressed, which was clinically consistent with his worsening condition. Biopsy specimen demonstrated nondestructive, diffusely infiltrating, large B-cell lymphoma, diagnosing LC. After the biopsy, corticosteroids were initiated, which dramatically alleviated his symptoms. Afterwards, he was treated by whole brain irradiation (total 36Gy) and discharged without noticeable deficits. Diagnosis of LC requires additional examinations generally not performed in the other white matter disorders. In suspected cases, biopsy should be performed to avoid deferring adequate cytostatic treatment.

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Acknowledgements

We greatly appreciate Drs. Yoichi Nakazato and Atsushi Sasaki (Department of Human Pathology, Gunma University Graduate School of Medicine, Gunma, Japan) for providing specimens immunohistochemically stained for Iba-1 and CD68, and their valuable scientific support.

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Authors and Affiliations

  1. Department of Neurosurgery, Shizuoka City Shimizu Hospital, Shizuoka, Japan

    Ryuichi Kanai & Koji Fujii

  2. Department of Pathology, Hachioji Medical Center, Tokyo Medical University, Tokyo, Japan

    Makoto Shibuya

  3. Department of Neurology, Shizuoka City Shimizu Hospital, Shizuoka, Japan

    Takashi Hata & Makoto Hori

  4. Department of Pathology, School of Medicine, Tokai University, Kanagawa, Japan

    Kenichi Hirabayashi

  5. Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan

    Tadashi Terada

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  1. Ryuichi Kanai
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  2. Makoto Shibuya
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  3. Takashi Hata
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  4. Makoto Hori
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  5. Kenichi Hirabayashi
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  6. Tadashi Terada
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  7. Koji Fujii
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Correspondence to Ryuichi Kanai.

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Kanai, R., Shibuya, M., Hata, T. et al. A case of ‘lymphomatosis cerebri’ diagnosed in an early phase and treated by whole brain radiation: case report and literature review. J Neurooncol 86, 83–88 (2008). https://doi.org/10.1007/s11060-007-9437-9

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  • DOI: https://doi.org/10.1007/s11060-007-9437-9

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