KEY POINTS
- This retrospective single-centre series included 6 consecutive patients with lacrimal gland adenoid cystic carcinoma treated in Beijing between 2012 and 2024, with contextual comparison against 22 SEER cases. Median age was only 31 years, and 4/6 patients had T3–T4 disease.
- Local disease was frequently extensive: orbital wall and extraocular muscle invasion each occurred in 50%, while optic nerve involvement was present in 66.7%. Four patients (66.7%) required orbital exenteration, and only 2/6 achieved R0 resection.
- All six patients received high-dose conformal RT, predominantly postoperatively, using VMAT in five cases and tomotherapy in one. Prescribed doses ranged from 66 to 69.96 Gy in 30–33 fractions, with QUANTEC-based ocular constraints and dedicated eye-protection measures.
- After a median 60-month follow-up, only 1/6 patients (16.7%) developed local recurrence. Five-year overall and disease-specific survival were both 75%, although the confidence interval was extremely wide (42.6–100%) because of the cohort size.
- Distant progression remained a major problem despite local treatment: 3/6 patients (50%) developed metastases, involving bone in two cases and lung in one, occurring at 10, 10 and 91 months after surgery.
- Treatment-related toxicity was predominantly grade 1–2. No severe ocular toxicity was reported, and long-term visual function was preserved in 5/6 patients (83.3%); the representative DVH in the paper shows a mean lens dose below 10 Gy and corneal dose below 40 Gy.
- In the exploratory combined regional-stage cohort of 17 patients, surgery plus RT was associated with numerically higher 5-year OS (64.8% vs 26.7%) and DSS (74.1% vs 26.7%) than surgery or RT alone. These were descriptive comparisons with very wide confidence intervals and should not be interpreted causally.
CLINICAL TAKEAWAY
For lacrimal gland ACC, aggressive surgery followed by approximately 66–70 Gy can provide durable local control while preserving ocular function when anatomy permits careful sparing. The dominant residual problem may be distant metastasis, but the six-patient dataset is far too small to define an optimal multimodality strategy.