Pediatric adenoid cystic carcinoma of the lacrimal gland treated with intra-arterial cytoreductive chemotherapy

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.