Abstract
Maxillary osteosarcoma is a rare malignant bone tumor of the craniofacial skeleton, accounting for a small proportion of head and neck sarcomas. Orbital involvement is exceptionally uncommon and poses significant diagnostic and therapeutic challenges. Early recognition and multidisciplinary management are critical for optimizing outcomes. A 41-year-old male presented with progressive nasal obstruction for one month and left eye vision loss for 20 days. Examination revealed a cystic mass in the left nasal cavity, a proliferative lesion on the hard palate, and left eye proptosis with complete visual loss. Imaging demonstrated a mass arising from the left maxillary sinus with erosion of the orbital floor and optic nerve compression. Initial biopsy suggested a chondro-osseous respiratory epithelial hamartoma. Due to the lesion's aggressive behavior and irreversible vision loss, the patient underwent left total maxillectomy with orbital exenteration and elective tracheostomy. Final histopathology confirmed conventional osteosarcoma, grade 3 (poorly differentiated/high-grade). Adjuvant chemotherapy with cisplatin and doxorubicin was administered. At the six-month follow-up, the patient remained disease-free, with no evidence of local recurrence or distant metastasis. Orbital extension of maxillary osteosarcoma is a rare and aggressive presentation. High clinical suspicion, comprehensive imaging, and histopathological confirmation are essential for diagnosis. Radical surgical resection, including orbital exenteration when indicated, combined with adjuvant chemotherapy, is critical for achieving oncologic clearance. Multidisciplinary management is key to improving outcomes in these complex cases.