Secretory Carcinoma of the Lacrimal Gland: A Rare Case Report.

Secretory carcinoma is a salivary gland malignancy that recapitulates secretory carcinoma of the breast, along with its shared ETV6-NTRK3 gene fusion. Characterization of histopathologic, immunohistochemical, and molecular genetic features of this neoplasm has led to reclassification of a heterogeneous group of salivary gland carcinomas as secretory carcinoma and to identification of this neoplasm in other gland-containing tissues. The authors describe a 52-year-old man who presented with a 2-week history of diplopia and a well-circumscribed right orbital mass. The tumor was resected via lateral orbitotomy approach. Pathologic evaluation demonstrated secretory carcinoma, previously not described in the main lacrimal gland. Recognition of lacrimal gland secretory carcinoma may lead to reappraisal of morphologically similar, but biologically heterogeneous lacrimal gland neoplasms, providing an insight into this tumor's clinical presentation and prognosis. Accurate diagnosis of this malignancy has important management and prognostic implications, particularly with emergence of targeted therapies.