Purpose Epithelial-myoepithelial carcinoma (EMC) is a low-grade salivary gland tumour which is very rarely reported in the lacrimal gland. Hybrid carcinomas such as EMC, defined by Seifert and Donath (1996),1 comprise two histological subtypes of carcinoma within the same topographic field. Hybrid tumours constitute <1% of all salivary gland tumours, with only eight previous documented cases of lacrimal gland EMC reported in the literature.2 The diagnosis of EMC is histological, demonstrating a biphasic dual morphology of luminal cuboidal ductal epithelial cells and outer myoepithelial cells with clear cytoplasm.1,2

Methods/Results We describe the case of a 92-year-old transgender man that presented with progressively worsening left-sided headaches, eye pain and with a clinical enlargement of the left lacrimal region. The patient underwent superior-lateral orbitotomy with excisional biopsy of the left lacrimal gland and was found to have EMC arising from a pleomorphic adenoma, which was subsequently managed with adjuvant rotation therapy.

Conclusions Patient prognostication and optimal management of these tumours provide a challenge for clinicians due to their inherent rarity. We describe the first case of EMC arising within a pleomorphic adenoma in the Australian population.