Disease-specific and overall survival for patients with lacrimal gland adenoid cystic carcinoma in recent decades

Abstract

Purpose To evaluate the presenting symptoms, and the overall survival (OS) and disease-specific survival (DSS) in patients with lacrimal gland adenoid cystic carcinoma (LGACC). Methods This retrospective single-centre cohort study included all consecutive patients with LGACC treated by the primary author from November 1998 through August 2024. Demographic data, presenting symptoms, the histological subtype of LGACC, type of surgical treatment, adjuvant radiotherapy or chemotherapy, T category at presentation and survival data were reviewed. Correlations between histological subtypes, T-category, type of surgery (eye sparing vs orbital exenteration), and DSS and OS were analysed. Results 52 patients had a median age of 43.5 years (range: 9–71). Pain was the most common symptom but was only present in 17 patients (33%), 26 patients (50%) had orbital exenteration and 25 (48%) had eye-sparing surgery. Eye-sparing surgery was offered to selected patients only since 2007. Overall, 47 patients (90%) had postoperative radiotherapy and 18 patients (35%) developed distant metastasis. At last contact, 14 patients (27%) had died of disease (median follow-up=60 months). The 5-year and 10-year DSS rates for patients who had eye-sparing surgery (87.1% and 76.2%, respectively) were better than for patients who had orbital exenteration (62.3% and 57.5%, respectively, p=0.049). There was a significant difference between the DSS for basaloid (solid) versus non-basaloid histologic subtypes with a worse survival for basaloid histology (p=0.00057). Conclusions Pain was present in only one-third of patients with LGACC. The 5-year and 10-year DSS and OS rates are better than previously reported ‘historic data’. Data are available upon reasonable request.