Rare eye cancer case: orbital exenteration keeps patient recurrence-free at 34 months

A 32-year-old Hispanic man with a six-year history of right orbital pressure that progressed to pain, double vision, and excessive tearing has been reported as a case of lacrimal gland adenoid cystic carcinoma, a rare and aggressive malignancy of the tear gland. The report, published in Frontiers in Ophthalmology, describes how the tumor was ultimately managed with orbital exenteration followed by adjuvant radiotherapy, with no evidence of local recurrence at 34 months of follow-up.

Lacrimal gland adenoid cystic carcinoma, abbreviated LGACC, is characterized by perineural invasion, late recurrence, and high mortality. According to the authors, no consensus treatment guidelines exist for this malignancy, and management strategies range from eye-sparing excision to orbital exenteration combined with chemotherapy and radiotherapy. The report comes from researchers at the Department of Ophthalmology and the Department of Pathology at Universidad de Puerto Rico Recinto de Ciencias Medicas in San Juan.

The patient’s clinical course began with a sensation of pressure in the right orbit that persisted for six years before progressing to pain, diplopia, and lacrimation. On examination, visual acuity was 20/20 in both eyes, but clinicians palpated a mass in the right upper eyelid and observed hypoglobus with inferonasal displacement of the globe, meaning the eye was pushed downward and toward the nose by the mass behind it.

Imaging identified a well-defined mass in the right lacrimal gland, and biopsy confirmed the diagnosis of adenoid cystic carcinoma.

Treatment planning presented significant challenges. Intra-arterial chemoreduction, a technique that delivers chemotherapy directly to the tumor’s blood supply, was not feasible in this case. The patient instead received three cycles of systemic cisplatin and doxorubicin, but the regimen produced no radiographic reduction in tumor size. With chemotherapy failing to shrink the lesion, the surgical team proceeded to right orbital exenteration, the removal of the orbital contents including the eye, followed by adjuvant radiotherapy.

At 34 months after treatment, the patient remains under multidisciplinary surveillance with no evidence of local recurrence. The authors emphasize that this outcome reflects one individual case and that the long follow-up required for this disease is far from over, given the well-documented tendency of LGACC to recur late, sometimes years after apparent remission.

The report’s central message is that LGACC is best managed with an individualized, interdisciplinary, and multimodal approach.

The authors call for further research to establish surveillance and management strategies for this malignancy. Until such evidence exists, they argue that long-term monitoring is essential for every patient, since the risk of late recurrence means that even years of disease-free follow-up do not equal cure. This single-patient case report adds to the limited published experience with LGACC but cannot by itself establish which treatment approach is superior.

Subject of Research: Management of a rare case of lacrimal gland adenoid cystic carcinoma treated with orbital exenteration and radiotherapy.

Article Title: Lacrimal gland adenoid cystic carcinoma: a rare case report

Article References: Ramírez Marquez, E., Garcia Forestier, M., Correa, M., & Campbell, J. (2026). Lacrimal gland adenoid cystic carcinoma: a rare case report. Frontiers in Ophthalmology, 6, Article 1936845. https://doi.org/10.3389/fopht.2026.1936845

Image Credits: AI Generated

DOI: 10.3389/fopht.2026.1936845

Keywords: lacrimal gland adenoid cystic carcinoma, orbital exenteration, case report, adenoid cystic carcinoma, radiotherapy, cisplatin, doxorubicin, ophthalmology, orbital tumor, perineural invasion, cancer surveillance, Lacrimal