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.