Examining progression of advanced lacrimal gland adenoid cystic carcinoma

Lacrimal gland adenoid cystic carcinoma (LGACC) is an uncommon malignancy with a variable prognosis. In this report, we present a case of LGACC whose locally advanced orbital disease was treated aggressively with several surgical and radiation therapies. Despite multiple local recurrences, the patient’s metastatic lung lesions remain radiographically stable over a decade after the initial onset of symptoms. A 42-year-old Black female presented in 2013 with worsening right frontal headaches, unilateral proptosis, and a palpable mass under the right temporal upper eyelid. Imaging demonstrated an orbital mass, which was subsequently grossly resected via a right cranio-orbital craniotomy in January 2014. Histopathology showed cribriform LGACC with perineural invasion and positive tumor margins. Ophthalmology consultation recommended orbital exenteration followed by adjuvant radiation therapy, but she declined. Shortly thereafter, a magnetic resonance imaging (MRI) detected an area of new enhancement in the anterior superolateral aspect of the right orbit. Orbital exenteration followed by chemotherapy was again recommended; however, the patient declined. Three years later, she reported an increase in proptosis and reduced corneal sensation and was found to have a recurrent orbital mass, but no further treatment was pursued despite recommendation for multimodal intervention. Six years after the resection, she agreed to enucleation, but not exenteration, due to tumor expansion and massive proptosis with exposure keratopathy. Over seven years after her initial presentation, surveillance imaging demonstrated radiographic evidence of lung metastases, though the patient remained asymptomatic from these lesions. In July 2025, imaging showed evidence of local recurrence, and the patient was again treated with gamma knife radiation, and shortly thereafter started on Lenvatinib and pembrolizumab. Presently, her progressive disease remains confined to the primary site and her clinical symptomatology is largely stable with low-dose daily dexamethasone. This case illustrates the heterogeneous clinical behavior of metastatic LGACC and raises the possibility that individualized management may allow deferral of toxic systemic therapy in select patients.