Abstract
OBJECTIVES: This study aims to analyze the behavior and treatment of adenoid cystic carcinoma (AdCC) in the pediatric and young adult population and to identify factors affecting overall survival (OS). MATERIALS AND METHODS: The study analyzed salivary gland malignancies in patients aged 0-21 with AdCC histology using the National Cancer Database from 2004 to 2018. RESULTS: A total of 72 patients (59.7% parotid, 36.1% submandibular, 1.4% sublingual, 2.8% unspecified) met criteria. Median age was 18 years [range: 0-21]. High-grade dysplasia was present in 67% of cases. Therapy consisted of primary surgery for all cases, regional lymph node dissection (LND) (74%), radiotherapy (71%), chemotherapy (8%), and chemoradiation (7%). The 5-year OS rate was 93.2% [95% confidence interval (CI): 86.9%-99.9%], respectively. Patients who underwent associated LND had improved OS (p = .0083, log-rank test) with a 5-year OS at 82.4% [95% CI: 66.1%-100%] versus 97.6% [95% CI: 93.0%-100%]. A significant difference in OS was found with unfavorable outcomes after positive marginal status: 5-year OS 84.1% [95% CI: 71.0%-99.7%] versus 100% [95% CI: 100%]; p < .001. Adjuvant therapy did not seem to impact the outcome. CONCLUSION: This study confirms that AdCC in children and young adults has an overall good prognosis despite frequent high grade. It suggests that cervical LND may be of importance, but the value of systematic adjuvant therapy is not confirmed. These findings emphasize the importance and relevance of population-based studies in shaping clinical practice and informing the design of future prospective investigations.