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Primary Vitreoretinal Non-Hodgkin Lymphoma

MedGen UID:
1684330
•Concept ID:
C4763388
•
Neoplastic Process
Synonym: Primary Vitreoretinal Lymphoma

Definition

An uncommon and potentially fatal intraocular non-Hodgkin lymphoma that involves the uvea, retina, vitreous body, and optic nerve. It is a subset of primary central nervous system non-Hodgkin lymphoma. The majority of cases are diffuse large B-cell lymphomas. [from NCI]

Professional guidelines

PubMed

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
A proposed protocol of intravitreal injection of methotrexate for treatment of primary vitreoretinal lymphoma.
Zhou N, Xu X, Liu Y, Wang Y, Wei W
Eye (Lond) 2022 Jul;36(7):1448-1455. Epub 2021 Jul 1 doi: 10.1038/s41433-021-01657-0. PMID: 34211136Free PMC Article
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma.
Carbonell D, Mahajan S, Chee SP, Sobolewska B, Agrawal R, Bülow T, Gupta V, Jones NP, Accorinti M, Agarwal M, Batchelor T, Biswas J, Cimino L, tenDam-van Loon NH, de-la-Torre A, Frenkel S, Pe'er J, Kramer M, Miserocchi E, Mochizuki M, Ness T, Rosenbaum JT, Sen HN, Simion M (VRL patient), Sitter H, Vasconcelos-Santos DV, Habot-Wilner Z, Coupland SE, Pulido JS, Smith J, Thorne JE, Zierhut M; Study Group for Vitreoretinal Lymphoma Diagnostics
Ocul Immunol Inflamm 2021 Apr 3;29(3):507-520. Epub 2021 May 19 doi: 10.1080/09273948.2021.1878233. PMID: 34009095

Recent clinical studies

Etiology

A proposed protocol of intravitreal injection of methotrexate for treatment of primary vitreoretinal lymphoma.
Zhou N, Xu X, Liu Y, Wang Y, Wei W
Eye (Lond) 2022 Jul;36(7):1448-1455. Epub 2021 Jul 1 doi: 10.1038/s41433-021-01657-0. PMID: 34211136Free PMC Article
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma.
Carbonell D, Mahajan S, Chee SP, Sobolewska B, Agrawal R, Bülow T, Gupta V, Jones NP, Accorinti M, Agarwal M, Batchelor T, Biswas J, Cimino L, tenDam-van Loon NH, de-la-Torre A, Frenkel S, Pe'er J, Kramer M, Miserocchi E, Mochizuki M, Ness T, Rosenbaum JT, Sen HN, Simion M (VRL patient), Sitter H, Vasconcelos-Santos DV, Habot-Wilner Z, Coupland SE, Pulido JS, Smith J, Thorne JE, Zierhut M; Study Group for Vitreoretinal Lymphoma Diagnostics
Ocul Immunol Inflamm 2021 Apr 3;29(3):507-520. Epub 2021 May 19 doi: 10.1080/09273948.2021.1878233. PMID: 34009095
Lenalidomide in combination with intravenous rituximab (REVRI) in relapsed/refractory primary CNS lymphoma or primary intraocular lymphoma: a multicenter prospective 'proof of concept' phase II study of the French Oculo-Cerebral lymphoma (LOC) Network and the Lymphoma Study Association (LYSA)†.
Ghesquieres H, Chevrier M, Laadhari M, Chinot O, Choquet S, Moluçon-Chabrot C, Beauchesne P, Gressin R, Morschhauser F, Schmitt A, Gyan E, Hoang-Xuan K, Nicolas-Virelizier E, Cassoux N, Touitou V, Le Garff-Tavernier M, Savignoni A, Turbiez I, Soumelis V, Houillier C, Soussain C
Ann Oncol 2019 Apr 1;30(4):621-628. doi: 10.1093/annonc/mdz032. PMID: 30698644
Optical coherence tomography manifestations of primary vitreoretinal lymphoma.
Saito T, Ohguro N, Iwahashi C, Hashida N
Graefes Arch Clin Exp Ophthalmol 2016 Dec;254(12):2319-2326. Epub 2016 Jun 1 doi: 10.1007/s00417-016-3395-x. PMID: 27251192
Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group symposium.
Chan CC, Rubenstein JL, Coupland SE, Davis JL, Harbour JW, Johnston PB, Cassoux N, Touitou V, Smith JR, Batchelor TT, Pulido JS
Oncologist 2011;16(11):1589-99. Epub 2011 Nov 1 doi: 10.1634/theoncologist.2011-0210. PMID: 22045784Free PMC Article

Diagnosis

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
Primary central nervous system lymphoma.
Schaff LR, Grommes C
Blood 2022 Sep 1;140(9):971-979. doi: 10.1182/blood.2020008377. PMID: 34699590Free PMC Article
How we treat primary central nervous system lymphoma.
Calimeri T, Steffanoni S, Gagliardi F, Chiara A, Ferreri AJM
ESMO Open 2021 Aug;6(4):100213. Epub 2021 Jul 13 doi: 10.1016/j.esmoop.2021.100213. PMID: 34271311Free PMC Article
Consensus Recommendations for the Diagnosis of Vitreoretinal Lymphoma.
Carbonell D, Mahajan S, Chee SP, Sobolewska B, Agrawal R, Bülow T, Gupta V, Jones NP, Accorinti M, Agarwal M, Batchelor T, Biswas J, Cimino L, tenDam-van Loon NH, de-la-Torre A, Frenkel S, Pe'er J, Kramer M, Miserocchi E, Mochizuki M, Ness T, Rosenbaum JT, Sen HN, Simion M (VRL patient), Sitter H, Vasconcelos-Santos DV, Habot-Wilner Z, Coupland SE, Pulido JS, Smith J, Thorne JE, Zierhut M; Study Group for Vitreoretinal Lymphoma Diagnostics
Ocul Immunol Inflamm 2021 Apr 3;29(3):507-520. Epub 2021 May 19 doi: 10.1080/09273948.2021.1878233. PMID: 34009095
Primary Vitreoretinal Lymphoma in HIV Infection.
Steffen J, Coupland SE, Smith JR
Ocul Immunol Inflamm 2021 Apr 3;29(3):621-627. Epub 2020 May 26 doi: 10.1080/09273948.2020.1751856. PMID: 32453669

Therapy

Evidence-based expert consensus on the management of primary central nervous system lymphoma in China.
Chen T, Liu Y, Wang Y, Chang Q, Wu J, Wang Z, Geng D, Yu JT, Li Y, Li XQ, Chen H, Zhuang D, Li J, Wang B, Jiang T, Lyu L, Song Y, Qiu X, Li W, Lin S, Zhang X, Lu D, Lei J, Chen Y, Mao Y
J Hematol Oncol 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7. PMID: 36176002Free PMC Article
Primary central nervous system lymphoma.
Schaff LR, Grommes C
Blood 2022 Sep 1;140(9):971-979. doi: 10.1182/blood.2020008377. PMID: 34699590Free PMC Article
How we treat primary central nervous system lymphoma.
Calimeri T, Steffanoni S, Gagliardi F, Chiara A, Ferreri AJM
ESMO Open 2021 Aug;6(4):100213. Epub 2021 Jul 13 doi: 10.1016/j.esmoop.2021.100213. PMID: 34271311Free PMC Article
Primary Vitreoretinal Lymphoma in HIV Infection.
Steffen J, Coupland SE, Smith JR
Ocul Immunol Inflamm 2021 Apr 3;29(3):621-627. Epub 2020 May 26 doi: 10.1080/09273948.2020.1751856. PMID: 32453669
Lenalidomide in combination with intravenous rituximab (REVRI) in relapsed/refractory primary CNS lymphoma or primary intraocular lymphoma: a multicenter prospective 'proof of concept' phase II study of the French Oculo-Cerebral lymphoma (LOC) Network and the Lymphoma Study Association (LYSA)†.
Ghesquieres H, Chevrier M, Laadhari M, Chinot O, Choquet S, Moluçon-Chabrot C, Beauchesne P, Gressin R, Morschhauser F, Schmitt A, Gyan E, Hoang-Xuan K, Nicolas-Virelizier E, Cassoux N, Touitou V, Le Garff-Tavernier M, Savignoni A, Turbiez I, Soumelis V, Houillier C, Soussain C
Ann Oncol 2019 Apr 1;30(4):621-628. doi: 10.1093/annonc/mdz032. PMID: 30698644

Prognosis

Primary central nervous system lymphoma.
Schaff LR, Grommes C
Blood 2022 Sep 1;140(9):971-979. doi: 10.1182/blood.2020008377. PMID: 34699590Free PMC Article
Primary Vitreoretinal Lymphoma in HIV Infection.
Steffen J, Coupland SE, Smith JR
Ocul Immunol Inflamm 2021 Apr 3;29(3):621-627. Epub 2020 May 26 doi: 10.1080/09273948.2020.1751856. PMID: 32453669
Gene expression profiling of primary vitreoretinal lymphoma.
Arai A, Takase H, Yoshimori M, Yamamoto K, Mochizuki M, Miura O
Cancer Sci 2020 Apr;111(4):1417-1421. Epub 2020 Mar 12 doi: 10.1111/cas.14347. PMID: 32056332Free PMC Article
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation.
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Lenalidomide in combination with intravenous rituximab (REVRI) in relapsed/refractory primary CNS lymphoma or primary intraocular lymphoma: a multicenter prospective 'proof of concept' phase II study of the French Oculo-Cerebral lymphoma (LOC) Network and the Lymphoma Study Association (LYSA)†.
Ghesquieres H, Chevrier M, Laadhari M, Chinot O, Choquet S, Moluçon-Chabrot C, Beauchesne P, Gressin R, Morschhauser F, Schmitt A, Gyan E, Hoang-Xuan K, Nicolas-Virelizier E, Cassoux N, Touitou V, Le Garff-Tavernier M, Savignoni A, Turbiez I, Soumelis V, Houillier C, Soussain C
Ann Oncol 2019 Apr 1;30(4):621-628. doi: 10.1093/annonc/mdz032. PMID: 30698644

Clinical prediction guides

Clinical features predictive of vision loss in patients with vitreoretinal lymphoma: a single tertiary center experience.
Kim M, Suh H, Park YG, Park YH
Sci Rep 2023 Mar 18;13(1):4478. doi: 10.1038/s41598-023-31414-0. PMID: 36934118Free PMC Article
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation.
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Lenalidomide in combination with intravenous rituximab (REVRI) in relapsed/refractory primary CNS lymphoma or primary intraocular lymphoma: a multicenter prospective 'proof of concept' phase II study of the French Oculo-Cerebral lymphoma (LOC) Network and the Lymphoma Study Association (LYSA)†.
Ghesquieres H, Chevrier M, Laadhari M, Chinot O, Choquet S, Moluçon-Chabrot C, Beauchesne P, Gressin R, Morschhauser F, Schmitt A, Gyan E, Hoang-Xuan K, Nicolas-Virelizier E, Cassoux N, Touitou V, Le Garff-Tavernier M, Savignoni A, Turbiez I, Soumelis V, Houillier C, Soussain C
Ann Oncol 2019 Apr 1;30(4):621-628. doi: 10.1093/annonc/mdz032. PMID: 30698644
Intraocular T-cell Lymphoma: Clinical Presentation, Diagnosis, Treatment, and Outcome.
Chaput F, Amer R, Baglivo E, Touitou V, Kozyreff A, Bron D, Bodaghi B, LeHoang P, Bergstrom C, Grossniklaus HE, Chan CC, Pe'er J, Caspers LE
Ocul Immunol Inflamm 2017 Oct;25(5):639-648. Epub 2016 Mar 22 doi: 10.3109/09273948.2016.1139733. PMID: 27002464
How we diagnose and treat vitreoretinal lymphoma.
Fend F, Ferreri AJ, Coupland SE
Br J Haematol 2016 Jun;173(5):680-92. Epub 2016 May 2 doi: 10.1111/bjh.14025. PMID: 27133587

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