This study explores the possibility that lacrimal secretory carcinoma exists, but may be misclassified as other tumor types.

Study design

Researchers retrospectively reviewed 350 lacrimal gland tumors and performed fluorescence in situ hybridization to confirm the presence of the ETV6-NTRK3 gene fusion, which is a characteristic feature of secretory carcinoma in other sites of the body. The presence of the genetic translocation was confirmed by next-generation sequencing.

Outcomes

The analysis identified a single case of secretory carcinoma in a young man who was treated with surgery alone and experienced no recurrence during 12 years of follow-up. The authors confirmed the diagnosis by demonstrating a specific immunohistochemical profile and the presence of the ETV6-NTRK3 gene fusion.

Limitations

The ETV6-RET fusion cannot distinguish between a primary lacrimal gland secretory carcinoma and a metastatic lesion because some metastatic lesions (eg, primary carcinoma of the breast) harbor the same translocation.

Clinical significance

Secretory carcinoma is a new lacrimal gland carcinoma that should be considered in the differential of low-grade lacrimal gland tumors. Demonstration of ETV6 rearrangement may distinguish secretory carcinoma of the lacrimal gland from other primary lacrimal gland tumors.