Spheno-orbital mesenchymal chondrosarcoma: a rare cause of exophthalmia

ABSTRACT

Intracranial primitive mesenchymal chondrosarcoma is a highly malignant and extremely rare tumour. Orbital chondrosarcomas was reported in only 18 cases until 2004. A 36-year-old woman presented with left painless exophthalmia with temporozygomatic tumefaction. This exophthalmia was progressive during 4 months, with decrease in visual acuity in left eye. Oculomotricity examination showed near ophtalmoplegia. Palpebral occlusion was impossible. Visual acuity was 10/10 on right eye and 6/10 in left eye. A temporal tumefaction was noted. This swelling was firm, non mobilisable and adhered to deep zygomatic area. Cranial and skull base CT scan showed a process occupying space lesion in the orbit and sphenotemporal area with extension in intracranial temporal fossa. Cranial and orbital MRI showed a hypo intense signal on T1- and hyper signal on T2- images. Cerebral angiography showed intense tumoural blush fed by external carotid and internal maxillary artery. Twenty-four hours before the surgery the internal maxillary artery was embolised. Surgery was performed using a left fronto temporal approach with lateral orbitotomy to access intraorbital tumoural extension and complete removal was performed. Histopathological specimens showed a mesenchymal chondrosarcoma with immunoreactivity to antivimentine body and no reactivity to leucocyte commun antigen, epithelial membrane antigen, cytokeratine, protein S 100 ordesmine. Postoperative period was unremarkable with regression of exophthalmia. The patient received chemotherapy and radiotherapy. After 12 months follow-up the patient is asymptomatic. Spheno-orbital chondrosarcoma is a rare cause of tumoural exophthalmia