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Life sciences · Journal article
Frontiers in Oncology · September 17, 2026
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Introduction Lacrimal gland adenoid cystic carcinoma (LGACC) is a rare, aggressive malignancy with limited epidemiological data, particularly from East Asian populations. Methods This retrospective single-center series describes the clinicopathological features, treatment patterns, and outcomes of six consecutive patients with pathologically confirmed LGACC treated at the Cancer Hospital, Chinese Academy of Medical Sciences between 2012 and 2024. Findings were placed in the context of 22 cases extracted from the SEER database (ICD-O-3 histology code 8200/3, primary site C69.6). Survival was estimated for the institutional cohort and, descriptively, for a combined regional-stage cohort. Results The median age at diagnosis was 31 years (range 26–59), with equal sex distribution. All tumors were regional-stage, and 66.7% were T3–4. All patients underwent surgery (orbital exenteration in 66.7%, globe-sparing resection in 33.3%; R0 margins in 33.3%) followed by high-dose conformal radiotherapy (66–70 Gy). One disease-related death occurred. Five-year overall survival (OS) and disease-specific survival (DSS) were both 75% (95% CI 42.6–100%). Local recurrence was observed in 16.7% and distant metastases in 50%. Treatment-related toxicity was mostly grade I–II. In a combined regional-stage cohort of 17 patients, surgery plus radiotherapy was associated with numerically higher 5-year OS (64.8% versus 26.7%) and DSS (74.1% versus 26.7%) compared with surgery or radiotherapy alone; these estimates are presented only for descriptive context and are limited by small numbers and wide confidence intervals. A sensitivity analysis excluding two SEER patients with unknown surgery status who received radiotherapy alone yielded consistent numerical trends. Chemotherapy was not used as primary or adjuvant therapy for the initial disease; it was given concurrently with radiotherapy for early postoperative residual disease in one patient and after delayed local recurrence in another. Discussion In this series of predominantly advanced LGACC, surgery followed by adjuvant high-dose conformal radiotherapy achieved favorable local control with predominantly mild toxicity. Combined SEER comparisons should be interpreted cautiously because of the small sample size.