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Background: Orbital adenocarcinoma usually is metastatic and rarely can be primary disease of the orbit with no clear standard of care. Herein, we report a case of primary apocrine adenocarcinoma in the orbit with invasion into lacrimal sac in a young patient. Case report: A 38-year-old male presented with enlarging lump in the left eye. Ocular imaging studies showed an orbital mass with invasion into the lacrimal sac and surrounding muscles. The lesion was found to be adenocarcinoma with apocrine differentiation in both pathology and immunohistochemistry. Full body imaging studies showed no extra ocular spread and no other tumors to suggest orbital metastasis. The patient was treated with orbital exenteration and was given adjuvant local radiotherapy and systemic chemotherapy because of positive margins and high risk pathological features. On serial exams over 5 years follow up, the patient was free of local recurrence or distant metastasis. Conclusion: Orbital adenocarcinoma can present as a primary orbital tumor in young adults originating from apocrine glands, and aggressive surgical resection can be lifesaving management.

Primary apocrine adenocarcinoma of the orbit with lacrimal sac invasion