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Updated: May 31, 2026

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Primary uveal diffuse large B-cell lymphoma: A population-based study.

Pierre Loap1, Youlia Kirova1, Rémi Dendale1

  • 1Department of Radiation Oncology, institut Curie, Paris, France.

Cancer Radiotherapie : Journal De La Societe Francaise De Radiotherapie Oncologique
|May 29, 2026
PubMed
Summary
This summary is machine-generated.

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Primary uveal diffuse large B-cell lymphoma is a rare, aggressive cancer. While outcomes are poor, some localized cases may achieve long-term remission, suggesting radiotherapy as a potential treatment.

Area of Science:

  • Ophthalmology
  • Oncology
  • Hematology

Background:

  • Primary uveal tract involvement by diffuse large B-cell lymphoma (DLBCL) is rare.
  • Ocular lymphomas are often indolent mucosa-associated lymphoid tissue (MALT) lymphomas with good prognoses.
  • Limited data exists on the characteristics of primary uveal DLBCL.

Purpose of the Study:

  • To evaluate the epidemiologic and clinical features of primary uveal diffuse large B-cell lymphoma.
  • To analyze survival outcomes in patients with this rare malignancy.
  • To compare primary uveal DLBCL with other ocular lymphomas.

Main Methods:

  • Retrospective analysis of the Surveillance, Epidemiology, and End Results (SEER) database (2000-2022).
  • Inclusion criteria: histologically confirmed DLBCL with primary localization in the choroid or ciliary body/iris.
Keywords:
CancerEpidemiologyEye cancerLymphomaLymphomeÉpidémiologieŒil

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  • Survival analysis using Kaplan-Meier estimates and Cox univariate models.
  • Main Results:

    • 51 cases (0.038%) of primary uveal DLBCL identified among 134,366 DLBCL cases.
    • Median age 65 years; predominantly Caucasian patients.
    • 5-year overall survival: 45.5%; 5-year cancer-specific survival: 58.4%.
    • Localized disease showed a 5-year cancer-specific survival of 59.4% with a plateau after 6 years.
    • No significant survival associations with clinical or treatment variables (radiotherapy, chemotherapy).

    Conclusions:

    • Primary uveal DLBCL is an ultra-rare, aggressive malignancy distinct from uveal MALT lymphoma.
    • Outcomes are generally poorer than MALT lymphoma, but localized disease may lead to long-term remission.
    • Radiotherapy might be a treatment option for selected patients; optimal management requires further investigation.
    • Further prospective studies are needed to define optimal management strategies.