Abstract: Adenoid cystic carcinoma of the lacrimal gland (LGACC) is one of the most common malignant epithelial tumors in the lacrimal gland region, characterized by aggressive local invasion, a high tendency for local recurrence, and a propensity for distant metastasis. Its occurrence and progression are closely associated with various molecular abnormalities, including dysregulation of normal signaling pathways caused by myeloblastosis (MYB）-related gene fusion and rearrangement, aberrant activation of the Notch signaling pathway that promotes tumor invasion, and increased molecular heterogeneity resulting from mutations in other oncogenes and related signaling pathways. Currently, surgical resection combined with postoperative adjuvant radiotherapy remains the mainstay of treatment for LGACC, and neoadjuvant intra-arterial chemotherapy may help improve local control and survival outcomes. With continuing advances in molecular biology and genomics, targeted therapies directed at key molecular abnormalities in LGACC have emerged as a promising research direction. 

Key words: Adenoid cystic carcinoma of the lacrimal gland, MYB proto-oncogene, Notch signaling pathway, Molecular targeted therapy