| Sumario: | Abstract: Sinonasal adenocarcinoma (SAC) is an exceptionally rare malignancy in the pediatric population, especially with extensive anterior cranial fossa involvement. Optimal management requires meticulous surgical planning and a multidisciplinary approach. We present a 16-year-old male with progressive nasal obstruction, epistaxis, facial swelling, anosmia, and proptosis. Imaging revealed a large, lobulated mass originating in the nasal cavity with intracranial and orbital extension. A combined transcranial and endoscopic trans-nasal resection was performed. Reconstruction of the skull base utilized a titanium mesh buttress with pericranial and fascia lata grafts. Histopathology confirmed low-grade, non-intestinal-type SAC. Two small residual lesions over the nasal dorsum were excised post-operatively. The patient subsequently received adjuvant radiotherapy. Follow-up imaging and endoscopic surveillance confirmed disease-free status at 6 months. This case highlights the complexity of pediatric SAC with skull base invasion. A combined surgical approach with innovative skull base reconstruction and timely adjuvant therapy can achieve effective disease control.
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