Malignant ectomesenchymoma of the paranasal sinuses with proptosis

Malignant ectomesenchymoma of the paranasal sinuses with proptosis. Problem: A 36-year-old woman presented with a feeling of pressure in the right orbit and proptosis of the right eye after a “common cold”. Methodology: Computed tomography (CT) of the maxillofacial region revealed, and endoscopy confirmed, a mass in the right ethmoid sinus, eroding the lamina papyracea and extending into the orbit. Pathology of multiple biopsies revealed a nasal neoplasm composed of neuroectodermal and mesenchymal neoplastic elements, suggestive of a malignant ectomesenchymoma (MEM). Magnetic resonance imaging was used for MEM staging. Computed tomography of the chest and abdomen show no evidence of distant metastases. Results: Due to the intracranial and intraorbital extension of the tumour, radical surgery was not an option. Appropriate chemotherapy (6 cycles of vincristine/ifosfamide/adriamycin and 2 cycles of vincristine/ifosfamide/cisplatin) and intensity-modulated radiation therapy were administered. Conclusion: Twenty-eight months after treatment, there was no evidence of residual or metastatic disease.