A retrospective case series of pediatric lacrimal gland tumors of epithelial origin.
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PURPOSE: Epithelial lacrimal gland tumors are rare in children. This study presents a retrospective case series of 4 pediatric patients with primary lacrimal gland tumors of epithelial origin. METHODS: A literature review was performed on previous cases of pleomorphic adenomas (PA) and adenoid cystic carcinomas (ACC). Pediatric patients with PAs and ACCs treated at Le Bonheur Children's Medical Center and St. Jude Children's Research Hospital between June 2018 and May 2025 were identified using SNOMED and CPT codes. RESULTS: One case of PA and three cases of ACC were identified through imaging and confirmed by pathology and genetic studies. Mean age at presentation was 14 years, and common initial findings were proptosis (50%) and hypoglobus (75%). All patients underwent surgical excision, and ACC cases received adjuvant proton beam therapy (100%) and chemotherapy (33%). At a mean follow-up of 5 years, all remained disease-free. CONCLUSIONS: This study highlights the clinical presentation, treatment, and outcomes of pediatric lacrimal gland tumors of epithelial origin. Complete surgical resection was curative for PA, while ACC required multimodal therapy. The use of adjuvant therapy in the cases of ACC appears to reduce recurrence rates and emphasizes the efficacy of combined treatment in pediatric patients.