Paediatric ocular adnexal lymphoma: a population-based analysis

Area of Science:

• Ophthalmology
• Pediatric Oncology
• Hematology

Background:

• Ocular adnexal lymphoma (OAL) is a rare malignancy affecting the tissues around the eye.
• Understanding the incidence and characteristics of pediatric OAL is crucial for diagnosis and treatment.
• Previous studies have primarily focused on adult populations, leaving a gap in knowledge regarding pediatric cases.

Purpose of the Study:

• To determine the incidence rates of OAL in children and adolescents.
• To describe the clinicopathological features of pediatric OAL.
• To analyze the survival outcomes for pediatric OAL patients.

Main Methods:

• Retrospective analysis of the Surveillance, Epidemiology, and End Results (SEER) database.
• Inclusion of patients aged 18 years or younger diagnosed with OAL between 1973 and 2015.
• Calculation of age-adjusted incidence rates and descriptive statistics for demographic and clinicopathological data.

Main Results:

• The incidence of pediatric OAL was found to be 0.12 per 1,000,000 population, with higher rates in males and Black individuals.
• Extranodal marginal zone lymphoma (EMZL) was the most frequent subtype (45.5%), followed by diffuse large B-cell lymphoma (DLBCL) (9.1%).
• Localized, low-grade conjunctival lymphomas were often treated with surgery and/or radiation, while high-grade and distant tumors received chemotherapy. Patients with DLBCL and anaplastic large cell lymphoma (ALCL) had significantly poorer survival.

Conclusions:

• Pediatric OAL is a rare condition, with EMZL being the most common subtype and typically associated with an excellent prognosis.
• Histological subtype is a critical predictor of outcome in pediatric OAL.
• Patients diagnosed with DLBCL and ALCL face a higher risk of cancer-specific mortality.