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Diagnosis and management of vitreoretinal lymphoma: present and future treatment perspectives

  • Forefront Review
  • Organizer: Koh-Hei Sonoda, MD
  • Published:
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Abstract

Intraocular lymphoma (IOL) is a rare malignant intraocular lymphocytic tumor that mimics uveitis. IOL is anatomically classified into vitreoretinal lymphoma (VRL) and uveal lymphoma; most IOLs are VRLs, while uveal lymphoma is rare. VRL is highly malignant, with 60%–85% of patients developing central nervous system (CNS) lymphoma; primary VRL (PVRL) is an ocular disease with poor prognosis. We aimed to review the management and both current and future treatments for VRL. VRL diagnosis is based on the results of cytopathological examination using vitreous biopsy. However, the positive ratio of vitreous cytology remains 29%–70%. A combination of adjunctive tests may improve diagnostic accuracy, but as yet no gold-standard regimen has been established. Methotrexate intravitreal injections are effective in controlling ocular lesions; however, this treatment allows CNS dissemination. The efficacy of systemic chemotherapy in suppressing CNS dissemination has been recently debated. A multicenter prospective study with a unified treatment protocol is required to clarify this issue. In addition, establishing a treatment protocol for elderly patients and those with poor general health is necessary. Moreover, relapsed/refractory VRL and secondary VRL are more difficult to treat than PVRL because they are prone to recurrence. Ibrutinib, lenalidomide with or without rituximab, and temozolomide are promising treatments for relapsed/refractory VRL. In Japan, Bruton’s tyrosine kinase (BTK) inhibitors have been approved for treating refractory CNS lymphoma. Furthermore, a randomized prospective study of tirabrutinib, a highly selective BTK inhibitor, is ongoing for evaluating the suppressing of CNS progression in patients with PVRL.

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Acknowledgments

This work was supported by Grant-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (KAKENHI No. 21K09681 and KAKENHI No. 21K09738). This work was supported by Grant-in-Aid for Scientific Research from the Japan Agency for Medical Research and Development (No. 22ek01909473s0503 and No. 22ek0109473s0503).

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  1. Department of Ophthalmology, Jichi Medical University Saitama Medical Center, 1-847 Amanuma, Ohmiya-ku, Saitama, Saitama, 330-8503, Japan

    Toshikatsu Kaburaki

  2. Department of Ophthalmology, The University of Tokyo Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan

    Toshikatsu Kaburaki & Kazuki Taoka

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  1. Toshikatsu Kaburaki
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  2. Kazuki Taoka
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Correspondence to Toshikatsu Kaburaki.

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T. Kaburaki, Grant-in-Aid for research (AbbVie, Santen, Eisai, Alcon, Daiichi-Sankyo), Financial support for research (HOYA, Senju, Kowa, Wakamoto), Lecture fee (AbbVie, Eisai, Santen, Novartis, Senju, Mitsubishi Tanabe, Wakamoto, Kowa, Ono); K. Taoka, Grant-in-Aid for research (Ono, Nipro), Lecture fee (Ono, Nippon Shinyaku, Takeda, Eisai, Chugai).

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Organizer: Koh-Hei Sonoda, MD

Corresponding author: Toshikatsu Kaburaki

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Kaburaki, T., Taoka, K. Diagnosis and management of vitreoretinal lymphoma: present and future treatment perspectives. Jpn J Ophthalmol 67, 363–381 (2023). https://doi.org/10.1007/s10384-023-00997-6

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