Background: Primary adenocarcinoma of the lacrimal gland is rare and usually appears in the literature as case reports. The authors retrospectively studied cases of primary adenocarcinoma of the lacrimal gland to determine which of the currently used methods was most effective in treating this tumor. Methods: The authors requested cases of primary adenocarcinoma of the lacrimal gland from orbital surgeons and ocular pathologists worldwide. Of the cases referred, 13 examples of primary adenocarcinoma of the lacrimal gland could be confirmed and studied for clinical features, management, and outcome. Results: The authors have follow-up data on all 13 patients. Six patients died of cancer, three are alive with recurrence, and four are alive and disease-free. Conclusion: Based on the results of this study, the authors ascertain that primary adenocarcinoma of the lacrimal gland is not inevitably fatal and should be treated with exenteration and adjuvant radiotherapy as soon as a histologic diagnosis is established. Shorter duration of symptoms before treatment appears to decrease the chance of metastases and increase the chance of long-term survival.
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