Aggressive syringomatous carcinoma of the orbit

A 68-year-old woman with an unusual tumor involving the right orbit presented with painful exophthalmos of the right eye. Excision biopsy of her right eyelid was performed. The specimen showed ductal differentiation with comma-like extensions identical to syringoma. Cellular atypia, an invasive growth pattern, and remarkable perineural invasion led to the diagnosis of syringomatous carcinoma. Magnetic resonance imaging showed that the tumor exhibited maxillary involvement, invading the supraorbital fissure and bone of the middle skull base. The patient underwent craniofacial resection and has been alive with no sign of recurrence for 2 years. This case suggests that wide excision of these tumors with a clear surgical margin using skull base surgical techniques may offer a good prognosis.