[Secretory carcinoma of the lacrimal gland]

Secretory carcinoma is a histological type mostly found in salivary gland tumors, but also in breast and skin tumors. These tumors share pathological characteristics and the presence of an ETV6-NTRK3 gene fusion. Secretory lacrimal gland carcinoma is even rarer. Our case is a middle-aged man who presented with a left orbital tumor with proptosis, diplopia and mass effect on the globe. Multimodal imaging revealed a heterogeneous tumor enhanced with gadolinium. There were no granulomatous foci. Total excision was preferred over diagnostic biopsy because of the intense vascularization of the tumor. Histopathology revealed a secretory carcinoma of the lacrimal gland. The FISH study showed ETV6 gene fission in 90% of enumerated cells. After consultation, it was decided not to irradiate because of the risk of xerophthalmia, cataract and meningioma. After eighteen months, follow-up imaging showed local recurrence, treated then by surgery and stereotactic radiation therapy. During follow-up, chemotherapy was added when a second recurrence at the orbital site occurred. To support our diagnostic approach, we found only two other cases of secretory carcinoma of the lacrimal gland in the medical literature, both treated by isolated excision. The characterization of histological subtypes of carcinomas has direct implication in the therapeutic approach with the use of targeted therapies. Thus, tyrosine kinase inhibitors can provide valuable options in the treatment of secretory carcinomas.