Squamous carcinoma ex pleomorphic adenoma of the lacrimal gland

We present a very rare case of squamous cell carcinoma (SCC) ex pleomorphic adenoma of the lacrimal gland. Our patient presented with a 12 month history of painful proptosis of his left eye associated with severe headache. Imaging showed a left lacrimal gland lesion with extensive orbital disease extending into lateral and superior rectus muscles, cavernous sinus and the greater wing of the sphenoid. A lacrimal gland biopsy showed a combination of small bland glandular structures and sclerotic, elastin-containing stroma showing that the SCC had arisen on a background of a probable pleomorphic adenoma. Treatment with cisplatin and 5-Fluorouracil proved efficacious with a significant reduction of orbital and post-orbital disease on interval scanning.