B-ENT

Malignant ectomesenchymoma of the paranasal sinuses with proptosis

1.

Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, Belgium

2.

Department of Otorhinolaryngology, Head and Neck Surgery, Sint-Jan General Hospital, Bruges, Belgium

3.

Department of Radiology,University Hospitals Leuven, Belgium

4.

Department of Radiation Oncology, University Hospitals Leuven, Belgium

5.

Department of Pathology, University Hospitals Leuven, Belgium

B-ENT 2011; 7: 201-204
Read: 757 Downloads: 583 Published: 14 February 2020

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.

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EISSN 2684-4907