Benign chondroid syringoma of the orbit: a rare cause of exophtalmos

We report a case of a 41-year-old woman with orbital CS. This tumor developed slowly over 8 years causing indolor, no axil, exophtalmos of the left eye. Computed tomography demonstrated an isodense intraorbital tumor with homogeneous enhancement without bony erosion. On Magnetic resonance imaging the tumor was isointense on T1-weighted imaging, slightly hyper intense on T2-weighted imaging, and enhanced after Gadolinium administration. The patient was operated via left lateral orbitotomy. At surgery the mass was well circumscribed, extraconal, very firm and did not invade or adhere to other structures. The tumor was removed in toto. The diagnosis was confirmed by histopathological examination, the lesion was nodular, and there was differentiation toward the adnexal ductal epithelium with chondromyxoid and adipocytic differentiation in the stroma. No recurrence was seen with one year follow-up.CS should be included in the differential diagnosis of intra-orbital tumors. Complete resection remains the best therapeutic option to prevent recurrence. Close followup is recommended because malignant transformation, although rare, is possible.Chondroid syringoma (CS) are rare mixed tumours of sweat-gland which were first described by Billroth in 1859, that have both a bening and malignant form. Hirsch and Helwig in 1961 [1] gave them the appellation CS, because of the presence of sweat gland elements which are set in a cartilaginous stroma. The commonest sites are the scalp, cheek, nose, upper lip, chin, and the forehead.CS of the orbit are extremely rare. In the better of our knowledege, only one case has been reported in the english litérature [2]. Here we report an unusual case of a patient who underwent complete resection of a intraorbital CS. The clinical presentation, histologycal findings and treatment, with review of the relevant literature, are discussed.A 41-year-old woman presented with a history of left exophtalmos for 8 years and deterioration of visual acuity i