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



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


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.


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).


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.


  1. Amit S, Purwar N, Agarwal A, Kanchan S. Primary orbital non-Hodgkin’s lymphoma. BMJ Case Rep. 2012.

  2. Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29:252–60.<252::AID-CNCR2820290138>3.0.CO;2-#

    Article  CAS  Google Scholar 

  3. Fitzpatrick PJ, Macko S. Lymphoreticular tumors of the orbit. Int J Radiat Oncol Biol Phys. 1984;10:333–40.

    Article  CAS  PubMed  Google Scholar 

  4. Nutting CM, Jenkins CD, Norton AJ, Cree I, Rose GE, Plowman PN. Primary orbital lymphoma. Hematol J. 2002;3:14–16.

    Article  PubMed  Google Scholar 

  5. Bouali S, Said IB, Yedeas MD, Abderrahmen K, Maatar N, Boubaker A, et al. Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study. Child’s Nerv Syst. 2016;32:437–40.

    Article  Google Scholar 

  6. Grasso D, Borreggine C, Ladogana S, De Santis R, Delle Noci N, Grilli G, et al. Sporadic Burkitt’s lymphoma/acute B-cell leukaemia presenting with progressive proptosis and orbital mass in a child. Neuroradiol J. 2016;29:231–5.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Gupta R, Yadav JS, Yadav S, Wadood A. Orbital involvement in nonendemic burkitts lymphoma. Orbit. 2012;31:441–5.

    Article  PubMed  Google Scholar 

  8. Kiratli H, Uzun S, Varan A, Akyüz C, Orhan D. Management of anaplastic lymphoma kinase positive orbito-conjunctival inflammatory myofibroblastic tumor with crizotinib. J AAPOS. 2016;20:260–3.

    Article  PubMed  Google Scholar 

  9. Hung IJ, Kuo TT, Sun CF. Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis. J Pediatr Hematol Oncol. 2020;21:38–41.

    Article  Google Scholar 

  10. Hassan AS, Elner VM. Orbital peripheral T-cell lymphoma in a child. Ophthal Plast Reconstr Surg. 2005;21:385–7.

    Article  PubMed  Google Scholar 

  11. Leidenix MJ, Mamalis N, Olson RJ, McLeish WM, Anderson RL. Primary T-cell Immunoblastic Lymphoma of the Orbit in a Pediatric Patient. Ophthalmology. 1993;100:998–1002.

    Article  CAS  PubMed  Google Scholar 

  12. Alkatan HM, Alrashed SH, Al-Rikabi AC, Al-Faky YH. Orbital T-cell lymphoma in youngest recorded patient - Early diagnosis, management, and successful outcome: A case report and review of the literature. J Med Case Rep. 2018;12:139.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Alford MA, Nerad JA, Conlan RM, Comito M, Giller RH. Precursor B-cell lymphoblastic lymphoma presenting as an orbital mass. Orbit. 1999;18:17–24.

    Article  PubMed  Google Scholar 

  14. Pomeranz HD, McEvoy LT, Lueder GT. Orbital tumor in a child with posttransplantation lymphoproliferative disorder. Arch Ophthalmol. 1996;114:1422–3.

    Article  CAS  PubMed  Google Scholar 

  15. Edelstein C, Shields JA, Shields CL, De Potter P, Eagle J, Hagstrom N. Non-african burkitt lymphoma presenting with oral thrush and an orbital mass in a child. Am J Ophthalmol. 1997;124:859–61.

    Article  CAS  PubMed  Google Scholar 

  16. King AJ, Fahy GT, Brown L. Null cell lymphoblastic lymphoma of the orbit [7]. Eye. 2000;14:665–7.

    Article  PubMed  Google Scholar 

  17. Chou YC, Shih IH, Yang CP, Kuo TT, Hong HS. Concurrent mycosis fungoides and precursor B cell lymphoblastic lymphoma in a 6-year-old child. Pediatr Dermatol. 2005;22:23–5.

    Article  PubMed  Google Scholar 

  18. Faridpooya K, Mulder MMS, Merks JHM, De Smet M, Pals ST, Saeed P. Precursor B lymphoblastic lymphoma of the orbit in a child: An unusual presentation of a non-Hodgkin lymphoma. Orbit. 2006;25:153–7.

    Article  CAS  PubMed  Google Scholar 

  19. Bhat SK, Shrestha SP, Barthakur R, Natarajan M. Atypical presentation and diagnostic pitfalls: A case of rapidly progressive bilateral proptosis in a child aged 18 months. Indian J Ophthalmol. 2008;56:68–70.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Marcotte EL, Ritz B, Cockburn M, Clarke CA, Heck JE. Birth characteristics and risk of lymphoma in young children. Cancer Epidemiol. 2014;38:48–55.

    Article  PubMed  Google Scholar 

  21. Olsen TG, Heegaard S. Orbital lymphoma. Surv Ophthalmol. 2019;64:45–66.

    Article  PubMed  Google Scholar 

  22. Triebwasser C, Wang R, DeWan AT, Metayer C, Morimoto L, Wiemels JL, et al. Birth weight and risk of pediatric Hodgkin lymphoma: Findings from a population-based record linkage study in California. Eur J Cancer. 2016;69:19–27.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Coupland SE, Krause L, Delecluse HJ, Anagnostopoulos I, Foss HD, Hummel M, et al. Lymphoproliferative lesions of the ocular adnexa: Analysis of 112 cases. Ophthalmology. 1998;105:1430–41.

    Article  CAS  PubMed  Google Scholar 

  24. Moustafa GA, Topham AK, Aronow ME, Vavvas DG. Paediatric ocular adnexal lymphoma: a population-based analysis. BMJ Open Ophth. 2020;5:000483.

    Article  Google Scholar 

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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).

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