Abstract

Ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma is uncommon in the pediatric population. Initial misdiagnosis is common and there is lacking consensus regarding the optimal approach to treatment. Herein, we report an atypical presentation of pediatric conjunctival MALT lymphoma and review the presentation and management of this rare condition.

Highlights

  • Whilst ocular involvement occurs in only one to two percent of extranodal non-Hodgkin Lymphomas (NHLs), mucosaassociated lymphoid tissue (MALT) lymphoma is the most common type of primary ocular adnexal lymphoma [1]

  • Primary ocular adnexal lymphoma is rare in children, and the majority of data regarding the condition is obtained from adult populations, [6]

  • Whilst various case series of ocular adnexal lymphomas include pediatric patients, they rarely specify details such as the patients’ presenting symptoms, diagnosis, or management approaches, which are critical to determining the course of pediatric conjunctival MALT lymphoma

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Summary

Introduction

Whilst ocular involvement occurs in only one to two percent of extranodal non-Hodgkin Lymphomas (NHLs), mucosaassociated lymphoid tissue (MALT) lymphoma is the most common type of primary ocular adnexal lymphoma [1]. MALT lymphoma most commonly involves the gastrointestinal tract, salivary gland, lung, and thyroid gland It typically affects patients in their fifth to seventh decades, with females at higher risk than males. A potential association between ocular MALT lymphoma and Chlamydia psittaci has been suggested but no definitive infectious etiology or association has been identified [4]. Conjunctival MALT lymphoma characteristically manifests as a painless fleshy-coloured “salmon-patch” lesion arising from the fornix [6]. In ambiguous cases, assessing responsiveness to a short course of antiallergenic treatment may aid in the diagnosis of patients presenting with a conjunctival “salmon-patch” lesion. We report an atypical case of conjunctival MALT lymphoma in a 15-year-old patient, presenting without the “salmon-patch” lesion that typically characterizes the condition. Duration of follow-up (in remission unless otherwise specified) 15 months Bilateral recurrence at 6 months 2.5 years

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