Sinonasal and Ventral Skull Base Neoplasms in the Pediatric Population: A Systematic Review and Meta-analysis

Background Sinonasal and ventral skull base neoplasms represent rare entities in the general population, and even rarer in the pediatric population with a reported incidence of only 0.03 to 0.05 per 100,000. Despite the close proximity of critical structures and resultant potential to profoundly impact quality of life and function, the literature regarding key features of these lesions is sparse due to their uncommon nature. Our objectives were to highlight trends in sinonasal and ventral skull base lesions in the pediatric population through a meta-analysis of the literature.

Methods A PubMed search was performed to identify case reports, case-series, and other analyses pertaining to both benign and malignant sinonasal and anterior skull base tumors in the pediatric population. Data describing epidemiology, presenting symptoms, tumor characteristics, site and stage, treatment modalities, outcome, and survival were analyzed.

Results A total of 89 potential publications were identified, including a preliminary analysis of 25 publications accounting for 581 patients over the past decade. Demographically, males represented 46.3% of all diagnosed cases, and diagnosis was at an average age of 9.8 years. The most common presenting symptoms included headaches (23%) followed by ophthalmologic complaints (16%). Among all malignant and benign tumors reported, rhabdomyosarcoma remained the most common etiology (27.6%). The nasal cavity was the most common primary site of disease (31.5%) and patients with stage III and IV diseases comprised the largest group (65.2%). Treatment modality differed widely depending on primary tumor site and stage as well as institutional practice; however, roughly half of the patients received single modality treatments (24.4 and 23.3% receiving either radiation therapy or endoscopic resection, respectively). Only 6.7% of reported cases experienced either local and/or metastatic disease at an average follow-up period of roughly 44 months.

Conclusion Pediatric sinonasal and ventral skull base tumors are rare entities that can often be overlooked or misdiagnosed resulting in late diagnosis, treatment, and potentially poorer outcomes. Therefore, a large analytical review of the current literature is warranted to highlight current trends and raise clinical awareness.