Vitreoretinal lymphoma

Bianka Sobolewska; Soon Phaik Chee; Fatma Zaguia; Debra Anne Goldstein; Justine R. Smith; Falko Fend; Manabu Mochizuki; Manfred Zierhut

doi:10.3390/cancers13163921

Vitreoretinal lymphoma

Bianka Sobolewska, Soon Phaik Chee, Fatma Zaguia, Debra Anne Goldstein, Justine R. Smith, Falko Fend, Manabu Mochizuki, Manfred Zierhut

College of Medicine and Public Health

Research output: Contribution to journal › Review article › peer-review

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Abstract

Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL.

Original language	English
Article number	3921
Number of pages	23
Journal	Cancers
Volume	13
Issue number	16
DOIs	https://doi.org/10.3390/cancers13163921
Publication status	Published - 2 Aug 2021

Keywords

CNS lymphoma
IL-10/IL-6 ratio
Methotrexate
MYD88
Vitreoretinal lymphoma

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title = "Vitreoretinal lymphoma",

abstract = "Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL.",

keywords = "CNS lymphoma, IL-10/IL-6 ratio, Methotrexate, MYD88, Vitreoretinal lymphoma",

author = "Bianka Sobolewska and Chee, {Soon Phaik} and Fatma Zaguia and Goldstein, {Debra Anne} and Smith, {Justine R.} and Falko Fend and Manabu Mochizuki and Manfred Zierhut",

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TY - JOUR

T1 - Vitreoretinal lymphoma

AU - Sobolewska, Bianka

AU - Chee, Soon Phaik

AU - Zaguia, Fatma

AU - Goldstein, Debra Anne

AU - Smith, Justine R.

AU - Fend, Falko

AU - Mochizuki, Manabu

AU - Zierhut, Manfred

PY - 2021/8/2

Y1 - 2021/8/2

N2 - Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL.

AB - Vitreoretinal lymphoma (VRL) is a rare variant of primary central nervous system lymphoma (PCNSL), mostly of diffuse large B cell lymphoma, which affects the retina and/or the vitreous with or without optic nerve involvement. The disease course is aggressive. Up to 90% of the patients develop central nervous system lymphoma within one year. The diagnosis of VRL is challenging due to nonspecific chronic and relapsing uveitis and is made by anterior chamber tab or vitreous aspirate biopsy. There is no established treatment protocol for VRL patients with bilateral involvement without CNS involvement. There are suggestions to use only intravitreal chemotherapy with methotrexate and/or rituximab. Alternatively, systemic high-dose MTX treatment or external beam radiotherapy is used. Further studies are needed to prove and confirm the prophylactic systemic therapy in preventing CNS involvement in limited VRL.

KW - CNS lymphoma

KW - IL-10/IL-6 ratio

KW - Methotrexate

KW - MYD88

KW - Vitreoretinal lymphoma

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U2 - 10.3390/cancers13163921

DO - 10.3390/cancers13163921

M3 - Review article

AN - SCOPUS:85111628281

SN - 2072-6694

VL - 13

JO - Cancers

JF - Cancers

IS - 16

M1 - 3921

ER -

Vitreoretinal lymphoma

Abstract

Keywords

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