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Corneal Lymphoma in Humans: First Population-Based Evidence of Occurrence and Clinical Characteristics
Pierre Loap1, Youlia Kirova2, Remi Dendale3
1Department of Radiation Oncology, Institut Curie, Paris, France; Proton Therapy Center, Institut Curie, Orsay, France; Laboratoire d'Imagerie Translationnelle en Oncologie, Institut Curie, Orsay, France.
Corneal lymphoma, a rare human cancer, has been identified for the first time. This study describes its characteristics and suggests a favorable prognosis, particularly with radiotherapy for localized disease.
Area of Science:
- Ophthalmology
- Oncology
- Hematology
Background:
- Ocular lymphomas are documented in various eye structures, but primary corneal lymphoma has not been reported in humans.
- Previous reports of corneal lymphoma are limited to animals, primarily horses.
Purpose of the Study:
- To investigate the existence of primary corneal lymphoma in humans.
- To characterize the epidemiologic and clinical features of this rare condition.
Main Methods:
- A population-based retrospective analysis of the SEER 17 registries (2000-2022) was performed.
- Cases coded as C69.1 (cornea) were identified, and demographic, pathological, treatment, and survival data were extracted.
- Incidence rates were age-adjusted, and survival analysis was conducted.
Main Results:
- Five cases of strictly localized corneal lymphoma were identified, with an incidence of 0.0028 per million person-years.
- The mean age at diagnosis was 75 years, with a 4:1 male predominance; three cases were mucosa-associated lymphoid tissue (MALT) lymphomas.
- Patients received local surgery and radiotherapy for unilateral disease; two deaths occurred, with three patients surviving, including two long-term survivors.
Conclusions:
- Human corneal lymphoma is exceptionally rare and shares a favorable prognosis with other ocular MALT lymphomas.
- Radiotherapy may achieve durable disease control in localized corneal lymphoma.
- These findings establish corneal lymphoma as a human entity and advocate for increased diagnostic awareness in ocular oncology.
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