Pal Soham S, Alam Md Shahid, Manikantan Kapila, Honavar Santosh
Department of Ophthalmology, Government Medical College, Jalgaon, Maharashtra, India.
Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Aditya Birla Sankara Nethralaya, Kolkata, West Bengal (A Unit of Medical Research Foundation, Chennai, India), India.
Indian J Ophthalmol. 2025 Oct 1;73(10):1399-1411. doi: 10.4103/IJO.IJO_2560_24. Epub 2025 Sep 25.
Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare orbital neoplasm. Despite this, it remains the most common epithelial malignancy of the lacrimal gland. It is notorious for high rates of local recurrence, metastases, and mortality despite aggressive management. The treatment protocols range from orbital exenteration or an excision biopsy followed by adjuvant therapy in the form of radiotherapy or chemotherapy or sometimes both. Older studies suggest that orbital exenteration may result in better local control of disease and possibly better long-term survival. This outlook has been challenged by recent studies which suggest multimodal treatment combining various treatment strategies aiming for globe salvage and better disease-free survival. The present review analyzes in detail the various clinical and histopathological features, staging of the disease, management modalities, and treatment outcomes of ACC of the lacrimal gland published over the past 30 years.
泪腺腺样囊性癌（ACC）是一种罕见的眼眶肿瘤。尽管如此，它仍是泪腺最常见的上皮性恶性肿瘤。尽管采取了积极的治疗措施，但它因局部复发、转移和死亡率高而声名狼藉。治疗方案包括眶内容剜除术或切除活检，然后辅以放疗或化疗形式的辅助治疗，有时两者兼用。较早的研究表明，眶内容剜除术可能会更好地局部控制疾病，并可能带来更好的长期生存率。最近的研究对这一观点提出了挑战，这些研究表明，采用多种治疗策略相结合的多模式治疗旨在挽救眼球并提高无病生存率。本综述详细分析了过去30年发表的关于泪腺腺样囊性癌的各种临床和组织病理学特征、疾病分期、治疗方式及治疗结果。
