Primary sebaceous carcinoma of the lacrimal gland.

Primary sebaceous carcinoma is an exceptionally rare tumor of the lacrimal gland and less than 10 cases have been so far published in the literature. Two adult patients aged 38 and 81 years, respectively, who suffered unilateral painful massive swelling of the lacrimal gland are described. The disease in the first patient initially manifested as ipsilateral parotid gland metastasis and the primary tumor could be detected 3 months later. Both tumors were rock hard and fixed on palpation, caused partial upper eyelid ptosis, displaced the globe anteromedially and impaired ocular motility. Magnetic resonance imaging studies showed mostly homogeneous, well-delineated and moderately contrast-enhancing lacrimal gland fossa tumors without bone destruction. The management consisted of incisional biopsy for the diagnosis, immediately followed by exenteration. The younger patient further underwent radical neck dissection, parotidectomy and orbital and neck radiotherapy, which provided him a 2-year disease-free survival. Histopathological examination showed poorly differentiated sebaceous carcinoma destructing completely the lacrimal gland with predominantly comedo pattern. Despite its rarity and lack of specific clinical and imaging signs, sebaceous carcinoma should be considered in rapidly evolving painful and hard lacrimal gland fossa tumors. Also noteworthy is the early propensity of this tumor to spread to regional draining lymph nodes and the parotid gland in particular.