Eneh Amaka, Parsa Kami, Wright Kenneth W, Strube Yi Ning J
Department of Ophthalmology, Hotel Dieu Hospital, Queen's University, Kingston, Ontario, Canada.
Cedars-Sinai Medical Center, Los Angeles, California, United States of America.
J AAPOS. 2015 Jun;19(3):272-4. doi: 10.1016/j.jaapos.2015.01.016. Epub 2015 Apr 15.
Adenoid cystic carcinoma (ACC) of the lacrimal gland is the most common primary malignant tumor of the lacrimal gland. It typically affects patients in the fifth decade of life and presents with rapid progression of pain, ptosis, motility disturbances, and sensory deficits of less than 1 year's duration. ACC is rare in children. Due to early, aggressive perineural and bony spread, there is a high risk of intracranial extension. Additionally, due to frequent hematogenous and lymphatic spread, there is a high rate of distant metastases even after treatment, which can occur late up to a decade or more. The currently accepted treatment for ACC is radical exenteration with orbitotomy and adjuvant chemotherapy and/or radiation therapy. Recently, intra-arterial cytoreductive chemotherapy (IACC) has been investigated as a neoadjuvant treatment modality. It has the advantage of increasing local concentration at the target tissue and decreasing systemic distribution. We report the first known pediatric case of ACC treated with IACC, followed by exenteration, radiation, and adjuvant intravenous chemotherapy. The patient was followed for 4 years, with no recurrence and no known complications.
泪腺腺样囊性癌（ACC）是泪腺最常见的原发性恶性肿瘤。它通常影响50岁左右的患者，表现为疼痛迅速进展、上睑下垂、眼球运动障碍和持续时间不到1年的感觉缺陷。ACC在儿童中罕见。由于早期、侵袭性的神经周围和骨质扩散，存在较高的颅内扩展风险。此外，由于频繁的血行和淋巴扩散，即使在治疗后也有较高的远处转移率，转移可能在十年或更长时间后才发生。目前公认的ACC治疗方法是行眶内容剜除术联合眼眶切开术以及辅助化疗和/或放疗。最近，动脉内减瘤化疗（IACC）已被作为一种新辅助治疗方式进行研究。它具有增加靶组织局部浓度和减少全身分布的优点。我们报告了首例已知的接受IACC治疗的儿童ACC病例，随后进行了眶内容剜除术、放疗和辅助静脉化疗。该患者随访了4年，无复发且无已知并发症。
