Surgical management and adverse factors for recurrence and long-term survival in adenoid cystic carcinoma patients with intracranial extension: A retrospective cohort study

Limited data regarding intracranial adenoid cystic carcinomas (ACCs) were available. The authors aimed to elaborate on the clinical characteristics, treatment strategy, and poor outcomes of the disease. Clinical data from all cases of intracranial ACCs treated at our institute were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. our series included 10 males and 9 females, with a mean age of 47.3 years. The most common presentations were cranial neuropathies (11 cases), followed by headache (4 cases), and nasal symptoms (4 cases). The radiologic spectrum for intracranial ACCs was broad. Gross total resection (GTR), subtotal resection, and partial resection were performed in 8 (42.1%), 6 (31.6%) and 5 (26.3%) patients, respectively. After a median follow-up of 35.0 months (range, 3.0-89.0 months), 8 patients (42.1%) died. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 89.2%, 57.3%, and 20.5% and 89.5%, 57.5%, and 32.9%, respectively. Although the differences were not significantly different, GTR and the use of radiotherapy and chemotherapy tended to improve the prognosis of the patients. Intracranial ACCs are rare neoplasms. GTR alone, if tolerable, is advocated as the optimal treatment for intracranial ACCs. Nevertheless, conservative excision may be preferred to avoid damage to vital structures. Radiotherapy and chemotherapy may be an alternative treatment. Intracranial ACCs tend to recur or metastasis within a few years of the initial surgery.