Pediatric laryngeal lymphoma: A systematic review of clinical presentation, management, and outcomes.
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AIM: Pediatric laryngeal lymphoma is a rare malignancy, with limited guidance available to inform diagnosis and management. This systematic review aims to synthesize published cases to characterize epidemiology, clinical presentation, diagnostic approaches, treatment strategies, and outcomes in children with primary or secondary laryngeal lymphoma. METHODOLOGY: This review was conducted in accordance with PRISMA guidelines. PubMed, Medline, and Embase searches were performed using the terms "larynx" OR "laryngeal" AND "lymphoma." Studies reporting pediatric patients (≤18 years) with histologically confirmed lymphoma involving the larynx were included. Exclusion criteria comprised reviews without new cases, non-lymphoma diagnoses, non-laryngeal involvement, adult cases, insufficient outcome data and animal studies. Data extracted included demographics, clinical features, investigations, lymphoma subtype, management, and outcomes. RESULTS: A total of 1196 studies were reviewed, with 17 meeting the inclusion criteria for final analysis. Eighteen patients were identified (median age 13 years; 66.7% male). Non-Hodgkin lymphomas accounted for 94.4% of cases, predominantly B-cell subtypes. The supraglottis was the most frequently affected subsite (50.0%). Disease was localised in two thirds of cases. Diffuse disease was more common in patients with B symptoms or immunodeficiency and was associated with significantly higher mortality (66.7%). Chemotherapy was the primary treatment modality, with radiotherapy or combined chemoradiotherapy used in selected cases. Surgical intervention was limited to biopsy or airway stabilization. Overall, 50.0% of patients achieved durable remission, while 38.9% died during follow-up. Locoregional recurrence occurred in 16.7% of cases, exclusively in patients with initially diffuse or transglottic disease. CONCLUSION: Pediatric laryngeal lymphoma requires a high index of clinical suspicion due to its rarity and variable presentation. Outcomes are generally favorable for localized disease, while diffuse involvement and immunodeficiency predict poorer prognosis. Early endoscopic evaluation, accurate histopathological diagnosis, multidisciplinary management, and long-term surveillance are essential to optimize outcomes.