Outcomes of Evolving Treatment Strategies for Lacrimal Gland Adenoid Cystic Carcinoma

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Advancements in surgical protocols have salvaged more eyes, and controlled more mestatises than older approaches, a study shows.

A multimodal sequential treatment of neoadjuvant chemotherapy, surgery, extended-field stereotactic external beam radiotherapy (EBRT), and adjuvant chemotherapy had the most success compared with 2 other treatment protocols in both eye salvage and metastasis control among patients with lacrimal gland adenoid cystic carcinoma (ACC), according to findings published in Ophthalmic Plastic and Reconstructive Surgery.

Patients (N=40) with lacrimal gland ACC who received care at an ocular oncology service in India between 1995 and 2015 were retrospectively evaluated for this study. ACC management changed in this time from surgery followed by EBRT (group 1; 1995-2002; n=12) to adjuvant chemotherapy, surgery, and EBRT (group 2; 2002-2006; n=8) to neoadjuvant chemotherapy, surgery, EBRT, and adjuvant chemotherapy (group 3; 2006 onward; n=20). Patient outcomes were evaluated through 2020 on the basis of treatment strategy.

The study population was comprised of 65% men or boys, aged mean 36 (range, 11-72) years, 95% had proptosis, and 67.5% had cribriform histopathology. Most patients underwent en bloc tumor excision (90%), the others underwent orbital exenteration (10%).

The average follow-up time was 58 (range, 29-180) months.

In groups 1, 2, and 3, local tumor recurrence was observed among 42%, 25%, and 15%; systematic metastasis among 67%, 13%, and 5%; and mortality among 42%, 13%, and 0%, respectively. The eye was salvaged among more of group 3 (95%) compared with groups 1 (66%) and 2 (87.5%) and vision salvage (≥20/40) occurred among 85% of groups 2 and 3 compared with 66% for group 1.

The 5-year failure free survival (FFS) for local recurrence was 76% and rates at 3 and 6 years did not differ significantly between groups (P =.59). The 5-year FFS for systemic metastasis was 84% overall. Stratified by group, FFS was greater among group 3 compared with group 1 (95% vs 46%; P =.02).

Treatment-related complications included tear supplementation (100%), blood transfusion (36%), granulocyte colony stimulation factor support (25%), radiation cataract (18%), and radiation retinopathy (12%).

A potential limitation of this study was the differing follow-up durations of the treatment groups.

These data indicated that the newer treatment strategy of neoadjuvant chemotherapy, surgery, EBRT, and adjuvant chemotherapy was more effective at reducing systemic metastasis and tended to reduce local recurrence and salvage eyes compared with earlier treatment strategies. A prospective, randomized trial would be needed to confirm these findings and help to develop evidence-based standardized protocols for the treatment of lacrimal gland ACC.


Manjandavida FP, Honavar SG, Mrthy R, et al. Does multimodal treatment improve eye and life salvage in adenoid cystic carcinoma of the lacrimal gland? Ophthalmic Plast Reconstr Surg. 2022;38(4):348-354. doi:10.1097/IOP.0000000000002108