Lacrimal gland adenoid cystic carcinoma: a rare case report

Lacrimal gland adenoid cystic carcinoma (LGACC) is a rare and aggressive malignancy characterized by perineural invasion, late recurrence, and high mortality. No consensus treatment guidelines exist, and management strategies range from eye-sparing excision to orbital exenteration combined with chemotherapy and radiotherapy. This report describes a 32-year-old Hispanic man with a six-year history of right orbital pressure that progressed to pain, diplopia, and lacrimation. Clinical examination revealed 20/20 visual acuity bilaterally, a palpable right upper eyelid mass, and hypoglobus with inferonasal globe displacement. Imaging identified a well-defined right lacrimal gland mass, and biopsy confirmed adenoid cystic carcinoma. Intra-arterial chemoreduction was not feasible; however, three cycles of systemic cisplatin and doxorubicin resulted in no radiographic tumor reduction. The patient subsequently underwent right orbital exenteration, followed by adjuvant radiotherapy, and remains under multidisciplinary surveillance, with no evidence of local recurrence at 34 months of follow-up. LGACC is best managed with an individualized, interdisciplinary, and multimodal approach. Given the lack of consensus guidelines and the risk of late recurrence, long-term monitoring is essential, and further research is required to establish surveillance and management strategies.