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Paediatric orbital lymphoma; a case series and review of the literature

Abstract

Objective

To describe a series of paediatric orbital lymphoma patients in a single tertiary referral centre.

Methods

A retrospective case-note search in the Oxford Eye Hospital of all patients under the age of 18 years with orbital lymphoma between 2010 and 2020. Demographic and clinical data were obtained, and a literature review was conducted.

Results

Five patients were identified with orbital lymphoma, mean age 48.2 ± 36 months (1–109 months), three were males. Clinical presentation included: ptosis, proptosis, lethargy, visual loss, and strabismus. Two patients had bilateral orbital disease and one patient was diagnosed within the first month of life. The tissue diagnosis revealed four cases of Burkitt’s lymphoma and one case of T- lymphoblastic lymphoma. Central nervous system (CNS) sampling was also positive in the four cases of Burkitt’s lymphoma. All patients were treated systemically for the lymphoma with chemotherapy. Complete remission was achieved in all cases post chemotherapy. Follow-up of 36.4 ± 18.9 months (10–61 months).

Conclusion

This is the largest published case series of paediatric orbital lymphoma. We described a patient diagnosed within the first month of life and we believe this to have developed intra-uterine. In this series, patients were younger, had more bilateral disease and had better outcome than previously described. This rare condition should be considered in any child with an orbital mass, at any age. When managed appropriately, good outcomes can be achieved.

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Fig. 1: Imaging and pathology of patient 1.
Fig. 2: Imaging of patients 3 and 4.
Fig. 3: Imaging of patient 5.

Data availability

All data in this manuscript is available in the John Radcliffe records under the laws of confidentiality.

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Acknowledgements

We are grateful to Dr. Debora Pehl (Oxford University Hospitals NHS Foundation Trust) who reviewed the biopsy, made the diagnosis and provided the images.

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All authors contributed in data collection and participated in the writing of the manuscript and review process

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Correspondence to Jonathan H. Norris.

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Keren, S., Lapira, M., McCallum, E. et al. Paediatric orbital lymphoma; a case series and review of the literature. Eye (2022). https://doi.org/10.1038/s41433-022-02266-1

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