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Original research
Paediatric ocular adnexal lymphoma: a population-based analysis
  1. Giannis A Moustafa1,2,
  2. Allan K Topham3,
  3. Mary E Aronow1,
  4. Demetrios G Vavvas1
  1. 1Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA
  2. 2Department of Ophthalmology, SUNY Downstate Health Sciences University, Brooklyn, New York, USA
  3. 3Coalition of Cancer Cooperative Groups Inc, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Demetrios G Vavvas; vavvas{at}meei.harvard.edu

Abstract

Objective To investigate the incidence, clinicopathological characteristics and survival of ocular adnexal lymphoma (OAL) in the paediatric population.

Methods and analysis In this retrospective case series, the Surveillance, Epidemiology and End Results database was accessed to identify individuals with OAL ≤18 years of age, diagnosed between 1973 and 2015. OAL located in the eyelid, conjunctiva, lacrimal apparatus and orbit were included. Main outcome measures were the age-adjusted incidence rates (IRs) per 1 000 000 population at risk (calculated for the period 2000–2015) and descriptive statistics of demographic and clinicopathological features.

Results The IR of paediatric OAL was 0.12 (95% CI 0.08 to 0.16) per 1 000 000. Males (0.15; 95% CI 0.10 to 0.22) and blacks (0.24; 95% CI 0.13 to 0.42) had a higher tendency for OAL development. A total of 55 tumours in 54 children were identified. The majority were localised (78.4%), conjunctival (49.1%) lymphomas. Extranodal marginal zone lymphoma (EMZL, 45.5%, n=25) was the most frequent subtype, followed by diffuse large B-cell lymphoma (DLBCL, 9.1%, n=5), B lymphoblastic lymphoma (7.3%, n=4), follicular lymphoma (5.5%, n=3), Burkitt lymphoma (5.5%, n=3), anaplastic large cell lymphoma (ALCL, 3.6%, n=2), small lymphocytic lymphoma (1.8%, n=1), diffuse large B-cell lymphoma, immunoblastic (1.8%, n=1) and panniculitis-like T-cell lymphoma (1.8%, n=1). Localised, low-grade, conjunctival lymphomas were frequently treated with complete excision with or without radiation, while high-grade and distant tumours usually received chemotherapy. Only 29.1% of paediatric OAL cases were treated with radiation. Three out of five (60%) patients with DLBCL died of lymphoma at a median follow-up of 21 (range 10–86) months, and 1 out of 2 (50%) patients with ALCL died of lymphoma at 23 months from diagnosis.

Conclusion OAL in the paediatric population is rare. The majority of OAL are EMZL and are characterised by excellent prognosis. The histological subtype was found to be the main predictor of outcome with cancer-specific deaths observed in patients with DLBCL and ALCL.

  • eye (globe)
  • neoplasia
  • pathology
  • child health (paediatrics)
http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/bmjophth-2020-000483

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    Footnotes

    • Contributors GAM conceptualised the study, searched the literature, analysed the data, wrote and revised the manuscript; AKT provided statistical feedback and revised the manuscript; MEA revised the manuscript; DGV conceptualised the study, revised the manuscript, earned funding for the study and supervised the whole work.

    • Funding This study was supported by the Yeatts Family Foundation (DGV); Monte J. Wallace (DGV); 2013 Macula Society Research Grant Award (DGV); a Physician Scientist Award (DGV); unrestricted grant from the Research to Prevent Blindness Foundation (DGV); National Eye Institute (NEI) R21EY023079-01/A1 (DGV); Loeffler Family Fund (DGV); R01EY025362-01 (DGV); ARI Young Investigator Award (DGV); Foundation Lions Eye Research Fund (DGV).

    • Competing interests DGV is consultant to Olix Therapeutics and Valitor and co-founder of The Therapeutics.

    • Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.

    • Patient consent for publication Not required.

    • Ethics approval This study was approved by the Massachusetts Eye and Ear Institutional Review Board & Partners Human Research (Protocol #: 2019P001227). Our study and data accumulation were in conformity with all country, federal or state laws, and the study adhered to the tenets of the Declaration of Helsinki.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement Data are available in a public, open access repository;