[Adenoid cystic carcinoma of the lacrimal gland]

A 38-year-old female presented with a left-sided peripheral N. VI-paresis and negative neuroradiologic work-up. After one year she developed an incomplete N. III paresis, numbness of her face correlating to N. V1 and V2, reduced lacrimal secretion as well as a palpable mass at the lateral orbital rim. Magnetic resonance imaging revealed an orbital tumor extending into the cavernous sinus. Biopsy disclosed an adenoid cystic carcinoma of the lacrimal gland with basaloid, cribriform and tubular pattern. Therefore, an exenteration with postoperative radiation therapy was performed. Immunohistochemical findings included positive reactions for keratin (KL-1 and AE 1 + 3), Vimentin and Egp 34. By determination of Ki 67 the proliferative activity of the tumor was found to be 15%. Our case report demonstrates that even in absence of an exophthalmus or pain a malignant orbital tumor may be assumed as the underlying cause for a combined monolateral oculomotor and trigeminal paresis. Immunohistochemical results support the notion, that the adenoid cystic carcinoma of the lacrimal gland may arise from precursor cells of the terminal duct system.