Background. Primary adenocarcinoma of the lacrimal gland is rare and u
sually appears in the literature as case reports. The authors retrospe
ctively 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 fo
r clinical features, management, and outcome. Results: The authors hav
e follow-up data on all 13 patients. Six patients died of cancer, thre
e are alive with recurrence, and four are alive and disease-free. Conc
lusion: 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 s
oon as a histologic diagnosis is established. Shorter duration of symp
toms before treatment appears to decrease the chance of metastases and
increase the chance of long-term survival.