Unveiling a distinctive presentation: Benign manifestation of primary ductal adenocarcinoma of the lacrimal gland.

Objective: To report the case of a man with well-demarcated cystic lesion in the right lacrimal gland, subsequently diagnosed as primary ductal adenocarcinoma of the lacrimal gland.

Methods: We report the clinical presentation, histopathologic, immunohistochemical features and treatment of this unique primary ductal adenocarcinoma of the lacrimal gland. Additionally, we analyze the factors contributing to the tumor's presentation and review the current literature regarding this uncommon neoplasm.

Results: The patient underwent total resection of the mass, revealing primary ductal adenocarcinoma of lacrimal gland. Histopathological examination revealed a solid nested, sieve-like (with observed acne-like necrosis), tubular, and small nested arrangement of the tumor. The tumor cells exhibited marked heterogeneity, and vascular tumor thrombi were observed in the stroma, along with evidence of neurological invasion. The immunophenotype analysis showed positivity for androgen receptor, GATA binding protein 3, gross cystic disease fluid protein 15 and cytokeratin-7, which is considered a ductal adenocarcinoma of lacrimal gland. Subsequently the patient underwent local radiotherapy. No local recurrences or metastases were reported during the 1-year follow-up.

Conclusions: Primary ductal adenocarcinoma of lacrimal gland is a rare tumor of the lacrimal gland. The manifestation of ductal adenocarcinoma of the lacrimal gland may include well-demarcated cystic lesions, indicative of distinct subtypes. Various treatment options are adapted to different subtypes of ductal adenocarcinoma of the lacrimal gland.