Pediatric Ocular Adnexal Lymphoma: Past, Present, and Future.
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PURPOSE: To characterize the clinical, imaging, and pathological spectrum of pediatric ocular adnexal lymphomas tumors (OAL), including both primary and secondary lesions, using contemporary World Health Organization Haematolymphoid (5th edition) classification. METHODS: This retrospective case series included patients <18 years with biopsy-proven lymphoma of the orbit or ocular adnexa managed by a single oculoplastic surgeon over a 30-year period. Demographic, clinical, imaging, histopathological, treatment, and outcome data were collected. Lesions were categorized as primary (confined to ocular adnexa with or without regional lymph nodes) or secondary (ocular adnexa involved as part of systemic lymphoproliferative disease). RESULTS: Eleven patients (median age 11 years; 6 male) were identified. Five (45%) had precursor B-cell lymphoblastic leukemia/lymphoma, and 3 (27%) had mature B-cell tumors, including 2 extranodal marginal zone lymphomas and 1 diffuse large B-cell lymphoma. Three (27%) had mature natural killer/T-cell neoplasms, including 1 primary cutaneous anaplastic large cell lymphoma, 1 anaplastic large cell lymphoma, and 1 extranodal natural killer/T-cell lymphoma, nasal type. Six patients (55%) presented with ocular adnexal involvement secondary to systemic disease, most commonly in the orbit. Periorbital swelling, proptosis, and palpable masses were the predominant ocular signs. Management ranged from surgical excision or topical interferon to systemic chemotherapy, radiotherapy, and hematopoietic stem-cell transplantation. At the last follow-up, outcomes varied with histological subtype and disease stage. CONCLUSIONS: Pediatric ocular adnexal lymphomas displays striking clinicopathologic heterogeneity, with a predominance of precursor lymphoblastic neoplasms and a notable proportion of high-grade mature B- and T/NK-cell lymphomas. Recognizing both primary and secondary lesions and applying accurate histopathological classification are critical for timely diagnosis and to guide modern targeted therapies. This series expands the evidence base for a rare disease and underscores the importance of integrating ophthalmic assessment with contemporary World Health Organization diagnostic frameworks.