Primary vitreoretinal lymphoma: A report from an international primary central nervous system Lymphoma Collaborative group symposium

Chi Chao Chan, James L. Rubenstein, Sarah E. Coupland, Janet L Davis, J. William Harbour, Patrick B. Johnston, Nathalie Cassoux, Valerie Touitou, Justine R. Smith, Tracy T. Batchelor, Jose S. Pulido

Research output: Contribution to journalArticle

182 Citations (Scopus)

Abstract

Primary vitreoretinal lymphoma (PVRL), also known as primary intraocular lymphoma, is a rare malignancy typically classified as a diffuse large B-cell lymphoma and most frequently develops in elderly populations. PVRL commonly masquerades as posterior uveitis and has a unique tropism for the retina and central nervous system (CNS). Over 15% of primary CNS lymphoma patients develop intraocular lymphoma, usually occurring in the retina and/or vitreous. Conversely, 65%-90% of PVRL patients develop CNS lymphoma. Consequently, PVRL is often fatal because of ultimate CNS association. Current PVRL animal models are limited and require further development. Typical clinical findings include vitreous cellular infiltration (lymphoma and inflammatory cells) and subretinal tumor infiltration as determined using dilated fundoscopy, fluorescent angiography, and optical coherent tomography. Currently, PVRL is most often diagnosed using both histology to identify lymphoma cells in the vitreous or retina and immunohistochemistry to indicate monoclonality. Additional adjuncts in diagnosing PVRL exist, including elevation of interleukin-10 levels in ocular fluids and detection of Ig H or T-cell receptor gene rearrangements in malignant cells. The optimal therapy for PVRL is not defined and requires the combined effort of oncologists and ophthalmologists. PVRL is sensitive to radiation therapy and exhibits high responsiveness to intravitreal methotrexate or rituximab. Although systemic chemotherapy alone can result in high response rates in patients with PVRL, there is a high relapse rate. Because of the disease rarity, international, multicenter, collaborative efforts are required to better understand the biology and pathogenesis of PVRL as well as to define both diagnostic markers and optimal therapies.

Original languageEnglish
Pages (from-to)1589-1599
Number of pages11
JournalOncologist
Volume16
Issue number11
DOIs
StatePublished - Dec 2 2011

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Lymphoma
Central Nervous System
Intraocular Lymphoma
Retina
T-Lymphocyte Gene Rearrangement
Posterior Uveitis
Optical Tomography
T-Cell Receptor Genes
Tropism
Lymphoma, Large B-Cell, Diffuse
Methotrexate
Interleukin-10
Neoplasms
Histology
Angiography
Radiotherapy
Animal Models
Immunohistochemistry
Recurrence
Drug Therapy

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Primary vitreoretinal lymphoma : A report from an international primary central nervous system Lymphoma Collaborative group symposium. / Chan, Chi Chao; Rubenstein, James L.; Coupland, Sarah E.; Davis, Janet L; William Harbour, J.; Johnston, Patrick B.; Cassoux, Nathalie; Touitou, Valerie; Smith, Justine R.; Batchelor, Tracy T.; Pulido, Jose S.

In: Oncologist, Vol. 16, No. 11, 02.12.2011, p. 1589-1599.

Research output: Contribution to journalArticle

Chan, CC, Rubenstein, JL, Coupland, SE, Davis, JL, William Harbour, J, Johnston, PB, Cassoux, N, Touitou, V, Smith, JR, Batchelor, TT & Pulido, JS 2011, 'Primary vitreoretinal lymphoma: A report from an international primary central nervous system Lymphoma Collaborative group symposium', Oncologist, vol. 16, no. 11, pp. 1589-1599. https://doi.org/10.1634/theoncologist.2011-0210
Chan, Chi Chao ; Rubenstein, James L. ; Coupland, Sarah E. ; Davis, Janet L ; William Harbour, J. ; Johnston, Patrick B. ; Cassoux, Nathalie ; Touitou, Valerie ; Smith, Justine R. ; Batchelor, Tracy T. ; Pulido, Jose S. / Primary vitreoretinal lymphoma : A report from an international primary central nervous system Lymphoma Collaborative group symposium. In: Oncologist. 2011 ; Vol. 16, No. 11. pp. 1589-1599.
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