Abstract
BACKGROUND: Juvenile nasopharyngeal angiofibroma (JNA) is a rare, highly vascular benign tumor primarily affecting adolescent males. It accounts for 0.05%-0.5% of head and neck tumors and is typically diagnosed in its early stages due to symptoms such as recurrent epistaxis and nasal obstruction. However, atypical presentations with minimal bleeding can delay the diagnosis, leading to advanced tumor progression. Surgical management of advanced-stage JNA is challenging due to its aggressive local invasion, high vascularity, and potential for intracranial extension. CASE PRESENTATION AND MANAGEMENT: We report the case of an 11-year-old male who presented with progressive right nasal obstruction, headaches, and only a few episodes of mild epistaxis (3-4 times per year) over three years. This atypical presentation led to a delayed diagnosis, allowing the tumor to progress to an advanced stage. Imaging studies, including contrast-enhanced CT and MRI, revealed a large lobulated, highly vascularized stage 4B JNA with extensive invasion into the pterygopalatine fossa, infratemporal fossa, orbit, and intracranial structures, abutting the cavernous sinus. Given the tumor's extensive involvement, a multidisciplinary approach was adopted. An endoscopic endonasal approach was chosen for tumor resection to minimize facial scarring, preserve normal anatomy, and reduce perioperative morbidity. A meticulous stepwise dissection was performed, addressing the tumor's extension into the orbit, infratemporal fossa, and skull base. Hemostasis was carefully managed, and no major intraoperative complications were encountered. RESULTS: The patient demonstrated an uneventful postoperative recovery, with no significant bleeding or cerebrospinal fluid (CSF) leakage. Postoperative imaging confirmed near-total resection, and follow-up evaluations at one, three, and 6 months showed no evidence of recurrence. The patient's nasal obstruction resolved, facial symmetry improved significantly, and no neurological deficits were observed. CONCLUSION: This case highlights the importance of considering atypical presentations of JNA, as minimal epistaxis can delay diagnosis and lead to extensive tumor spread. Endoscopic surgical techniques provide an effective and minimally invasive alternative for managing advanced-stage JNA, offering superior cosmetic and functional outcomes while reducing perioperative risks. A multidisciplinary approach, integrating advanced radiological imaging and precise surgical planning, remains crucial in optimizing patient outcomes.