RESUMO
BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare malignant tumor. Moreover, only 3 cases of LGFMS originating from the nasal cavity and/or paranasal sinuses have been published so far. METHODS: Two patients with histologically confirmed LGFMS of the maxillary sinus were primarily treated by open and endoscopic surgery. In one case adjuvant radiotherapy was indicated because of the uncertainty of the surgical margins. RESULTS: Both surgeries were technically demanding and accompanied by significant bleeding from the tumors. Despite the extensive interventions there were no postoperative complications and no significant morbidity of the patients. There is no evidence of the disease 148 and 65 months after the treatment. CONCLUSIONS: Regardless of the excellent post-treatment results, based on our experience, preoperative embolisation of the tumor should be considered prior to the surgical resection.