Chemotherapy Approach Improves Lacrimal Gland Adenoid Cystic Carcinoma Survival

Medical Team Preparing Patient For MRI Scan
A medical team working together preparing both the machine and the patient for an MRI scan.
Investigators review neoadjuvant intra-arterial cytoreductive chemotherapy for orbital malignancy.

An extended follow-up of patients with lacrimal gland adenoid cystic carcinoma (LGACC), published in the American Journal of Ophthalmology, found that neoadjuvant intra-arterial cytoreductive chemotherapy (IACC) improved overall survival and decreased disease relapse at up to 15 years. 

The retrospective case-series review was conducted at the University of Miami-Miller School of Medicine. Patients (N=19) with LGACC who received neoadjuvant IACC, orbital exenteration, chemoradiotherapy, and adjuvant intravenous chemotherapy or conventional bimodal therapy since 1967 were contacted by telephone in January 2021 and asked about their disease-free survival. The referring ophthalmologist or medical oncologist of the patients were contacted about data on medical complications. Clinical outcomes were evaluated on the basis of whether patients had an intact (n=8) or lacked an intact (n=11) lacrimal artery.

The investigators took into account patients with (5 men, age range 29-73 years) and without (6 men, age range 20-64 years) intact lacrimal arteries. Among the intact artery cohort, 88% had advanced T4a-c tumors and 3 of these patients died. Two deaths were unrelated with disease, and 1 occurred after a recurrence at 74 months. At 15 years, 87.5% of this group had survived. The longest disease-free survivor had survived for 32.4 years after their T4c disease was determined inoperable.

For the cohort without an intact artery, 90.9% had T3 or more progressed disease. Chemotherapy was delivered intra-arterially for these patients. Seven patients in this group had died, 4 due to metastatic LGACC who presented with T4b-c disease. At 15 years, 34.1% of the group had survived.

In general, an intact lacrimal artery associated with improved all-cause survival (P =.042) and recurrence-free survival (P =.017). Among the subset of patients with T3 tumors or more advanced disease, an intact lacrimal artery remained associated with superior overall survival (P =.025).

At resection, 2 with intact arteries and 6 without intact arteries had positive tissue margins. Positive margins were associated with increased risk for disease-specific mortality by a factor of 8 (P =.043).

This study was limited by its small sample size and nonrandomized, retrospective design.

“This report describes the outcome of high risk and advanced tumor stage population of patients with LGACC treated by a trimodal therapy with neoadjuvant intra-arterial chemotherapy as the core element of the strategy,” investigators explain. “The report documents lengthy follow-up data to a cumulative duration of 15 years associated with excellent survival outcomes, which we attribute to the integration of neoadjuvant intra-arterial and adjuvant intravenous chemotherapy as the third arm of the trimodal strategy. An intact lacrimal artery appears central to IACC delivery for an optimal survival outcome.”


Tse DT, Benedetto PW, Tse BC, Feuer WJ. Neoadjuvant intra-arterial cytoreductive chemotherapy for lacrimal gland adenoid cystic carcinoma: a long-term follow-up study of a trimodal strategy. Am J Ophthalmol. Published online April 2, 2022. doi:10.1016/j.ajo.2022.03.027