Abstract
BACKGROUND: To present clinical presentations, histological-pathological patterns, clinical T stage, divergent treating methods, and outcomes of primary adenoid cystic carcinoma (ACC) of the lacrimal gland in a Chinese population. METHODS: This case series included patients with primary lacrimal gland adenoid cystic carcinoma treated at a Chinese hospital between 2003 and 2014. An exploratory multivariate Cox regression analysis was performed to evaluate the prognostic impact of clinical T-stage. Subsequently, Kaplan-Meier survival analysis was conducted, stratifying patients by T-stage (T4 vs. T1-T3) and by surgical approach among T4 patients, to assess the influence of tumor stage and surgical management on disease-free survival outcomes. RESULTS: A total of 38 patients included 16 men and 22 women with a median age of 46.3 years were enrolled. Sixteen patients (42.11%) had local recurrence, while nineteen patients (50%) had distant metastasis at the time of presentation. Twelve patients (31.58%) were in T1-T3 stage and twenty-six (68.42%) were in T4 stage. Nineteen patients (73.08%) in the T4 stage exhibited a predominantly solid-basaloid pattern, and only three (25%) in T1-T3 stage had a predominantly solid-basaloid pattern histological pattern. Median DFS for the entire cohort was 29.0 months (95% CI, 19.0-39.0), and median survival after metastasis was 7.0 months (95% CI, 3.0-9.0). After adjustment for covariates, T4 stage remained independently associated with significantly shorter DFS (HR = 4.46, 95% CI: 1.40-14.21, P = 0.011). A significant difference in DFS was observed between the T1-T3 and T4 groups (log-rank P = 0.003). Meanwhile, no significant difference in disease-free survival was observed between T4 patients undergoing globe-preserving surgery and eye-sparing approaches (log-rank P = 0.297). CONCLUSIONS: In this Chinese cohort, the solid-basaloid pattern correlated strongly with advanced T4 disease and aggressive behavior. Kaplan-Meier and multivariate Cox analyses consistently demonstrated that T4 stage was independently associated with significantly poorer DFS. For T4 patients, DFS did not differ significantly between exenteration and globe-preserving surgery when combined with radiotherapy, suggesting that eye-sparing approaches may be viable in advanced cases.