Intraocular Lymphomas

  • Kaustubh Mulay
  • Santosh G. Honavar
  • Santosh U. Kafle
  • Sarah E. CouplandEmail author


Lymphoma is a malignant neoplasm derived from monoclonal proliferations of B- or T-lymphocytes and rarely, natural killer (NK) cells. Ophthalmic involvement is relatively rare and may involve the ocular adnexa, orbit or intraocular structures. The intraocular lymphomas represent about 0.01% of ophthalmic diseases [1, 2]. They may arise as primary tumours within the eye, either within the vitreoretinal or uveal tract, or occur as secondary manifestations of a systemic Non-Hodgkin lymphoma (NHL). Vitreoretinal lymphomas (VRL) often occur in association with central nervous system (CNS) disease and are aggressive tumours. In contrast, primary uveal lymphomas tend to be low-grade B-NHL, and have a good prognosis. Secondary lymphomas represent an intraocular manifestation of a systemic NHL, and commonly occur in the choroid. Given their rarity and ability to masquerade clinically as inflammatory processes, the clinical diagnosis of intraocular lymphoma (both VRL and uveal lymphoma) is generally challenging, and typically requires a vitrectomy/chorioretinal biopsy for histopathological confirmation. Treatment of VRL is controversial and varies between centres, particularly in the absence of a concurrent CNS disease [2]. Although the earlier recognition of VRL has improved in recent times, the prognosis remains poor. Here, we summarize the available literature on both VRL and uveal lymphomas.


Vitreoretinal lymphoma Uveal lymphoma Primary intraocular lymphoma 


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© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  • Kaustubh Mulay
    • 1
  • Santosh G. Honavar
    • 2
  • Santosh U. Kafle
    • 3
    • 4
  • Sarah E. Coupland
    • 4
    Email author
  1. 1.National Reporting Centre for Ophthalmic Pathology (NRCOP), Centre For SightHyderabadIndia
  2. 2.Department of Ophthalmic and Facial Plastic Surgery and Ocular Oncology, Centre For SightHyderabadIndia
  3. 3.Department of Ophthalmic Pathology and Laboratory MedicineEastern Regional Eye Care Program, Biratnagar Eye HospitalBiratnagarNepal
  4. 4.Department of Molecular and Clinical Cancer MedicineInstitute of Translational Medicine, University of LiverpoolLiverpoolUK

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