Primary ductal adenocarcinoma of the lacrimal gland: case report.

A 78-year-old male patient presented with double vision, painless palpable mass under the right superolateral orbital rim, downward displacement and restricted adduction of the right eye. His visual acuity was 20/50 OD and 20/20 OS. Hertel exophthalmometry was 21 mm OD and 17 mm OS. Computed tomographic scans showed an infiltrative orbital mass with ill-defined, irregular margins, involving the lacrimal gland and the lateral rectus muscle. The patient underwent an anterior transcutaneous transseptal orbitotomy with incisional biopsy and surgical debulking. Histopathologic evaluation revealed primary ductal adenocarcinoma of the lacrimal gland. Following the metastatic work up, he underwent an eyelid-sparing orbital exenteration. Microscopically, the tumor elements were characterized by large polygonal cells with vesicular nuclei, prominent nucleoli and amphophilic cytoplasm. The tumor components comprised duct-type structures with papillary and cribriform patterns, surrounded by prominent basement membrane. The tumor cells were positive for cytokeratin-7, matrix metalloproteinase (MMP)-2, MMP-9, MMP-13 and proto-oncogene Her-2/neu, but negative for cytokeratin-5, cytokeratin-20, p63, prostate-specific antigen, S-100 protein and thyroid transcription factor. These histopathologic findings were compatible with poorly differentiated ductal adenocarcinoma of the lacrimal gland, T3N0M0. Twenty-four months after orbital exenteration, the patient was diagnosed with ipsilateral parotid gland and cervical lymph node metastases and died of disease.