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

Abstract

Chondroid syringoma (CS) of the orbit is an extremely rare benign neoplasm. To the best of our knowledege, this is the second case reported in the english litérature.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.

Figure 1

(A): Cranial CT scan demontrated an intra-orbital tumor, well circumscribed with homogeneous enhancement without bone erosion. (B, C and D): MRI showed a round mass that was extraconal, isointensity on T1-weighted imaging, slightly hyperintensity on T2-weighted imaging and enhanced after intravenous contrast administration.

Figure 2

(a): gross pathologic analysis revealed well-encapsulated tan tissue measuring 2 × 3 × 1,5 cm. No areas of hemorrhage or necrosis were noted. (b): a photomicrograph of the surgical specimen shows an encapsulated proliferation of nonbranching ducts set in chondromyxoid stroma. (hematoxylin and eosin stain, ×100). (c): postoperative CT image schows complete removal with good orbital reconstruction.