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Aims This study aimed to evaluate the expression of nuclear survivin in adenoid cystic carcinoma ( ACC) of the lacrimal gland and to determine if this expression is associated with histopathological features, markers of apoptosis and proliferation or clinical outcomes. Methods and results Immunohistochemical staining for survivin, p53, Ki-67 and Bcl-2 was analyzed in 55 cases of ACC of lacrimal gland. Thirty-one cases (56.3%) expressed nuclear survivin. All cases expressed p53, Ki-67 and Bcl-2. Eleven cases (35.5%) had a high nuclear survivin score ( NS- SCORE) and 20 cases (64.5%) had a low NS- SCORE. Cases with a high NS- SCORE had a shorter progression-free survival ( PFS) ( P < 0.0001), higher expression of Ki-67 ( P < 0.005) and a solid tumour pattern >30% ( P < 0.005). Conclusion Nuclear expression of survivin impacts prognosis significantly and is thus a promising prognostic marker in ACC of the lacrimal gland. [ABSTRACT FROM AUTHOR]