BENIGN CHONDROID SYRINGOMA OF THE ORBIT: A CASE REPORT.

Mixed tumors of the skin have a dual component, one epithelial and the other mesenchymal, closely resembling mixed tumors of the salivary or lacrimal glands. In 1961 the term “myxoid tumor of the skin” was replaced by “chondroid syringoma (CS)” in order to differentiate the common mixed tumors of the salivary or lacrimal glands from those, much rarer, of the skin, originating in the sweat glands.

CS is localized mainly to the scalp, face and neck.

This tumor can also occur in the orbit, but its prevalence is minimal. Only one case of intra-orbital chondroid syringoma has been identified in the literature.

We report an unusual case of a patient presenting an intra-orbital chondroid syringoma evolving for 8 years, who consults for a unilateral exophthalmos with reduced left visual acuity.

It’s a 41-year-old patient, with no notable pathological history, who consults for an exophthalmos of the left eye which has progressed for 8 years associated with a decline in visual acuity installed for 8 months.

The ophthalmologic examination of the left eye finds:

Painless, non-pulsatile, irreducible downward oblique proptosis with slightly limited abduction.

A visual acuity of 8/10 that cannot be improved.

An intraocular pressure at 16mmhg.

And a normal fundus.

The examination of the right eye is strictly normal with visual acuity of 10/10.

Examination of the visual field showed a decrease in sensitivity and limited narrowing of the visual field temporally in the left eye.

An orbital-cerebral computed tomography scan showed a well-circumscribed superolateral left orbital tumor without bone erosion. Magnetic resonance imaging revealed an extra-conical round mass with exophthalmos and medial optic nerve repression.

Our patient underwent surgery on her tumor by lateral orbitotomy with complete ablation. The diagnosis of Chondroid syringoma was confirmed by histopathology.

Chondroid syringomas (CS) are rare tumors of the skin appendages, slow growing, their preferred sites remain the head and the neck region.

Their orbital situation is exceptional, only one case has been listed in all the literature..

From a histological point of view, the characteristic epithelial and mesenchymal components allow the diagnosis to be made.

The radiologic signs of CS are not as suggestive as the histologic findings. MRI features are nonspecific, but they can accurately describe the anatomical extent and identify tissue of origin and depth of relative invasion to adjacent structures.

The differential diagnosis in our case is with other benign orbital lesions.

The treatment of choice remains complete excision. In our patient, the tumor was completely excised without recurrence after a follow-up of one year.

Chondroid syringomas remain an extremely rare entity but not excluded in the diagnosis of intra-orbital tumors. Complete excision is the most effective treatment method. Although malignant forms of these tumors and cases of recurrence are very rare, long-term follow-up remains an obligation.