Bin-Alamer Othman, Haider Ali S, Chaudhary Adhiraj, Balasubramanian Kishore, Breeding Tessa, Palmisciano Paolo, Haider Maryam, Cohen-Gadol Aaron A, Ahmadieh Tarek Y El, Yu Kenny
College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
King Abdullah International Medical Research Center, Riyadh, Saudi Arabia.
Cancer Diagn Progn. 2022 Sep 3;2(5):503-511. doi: 10.21873/cdp.10134. eCollection 2022 Sep-Oct.
BACKGROUND/AIM: To systematically review the patient characteristics and management approaches of adenoid cystic carcinoma (ACC) infiltrating the skull base.
According to PRISMA guidelines, PubMed, Scopus, and Cochrane were searched to retrieve studies reporting management protocols and survival outcomes of patients with skull base ACCs. Patient characteristics, management strategies, and outcomes were investigated.
The review encompassed 17 studies involving 171 patients, with a female predominance (57.9%) and a mean age of 49±7.12 years. ACCs mostly infiltrated the paranasal sinus (22.2%), cavernous sinus (8.8%), and nasopharynx (7.1%). Perineural invasion was reported in 6.4% of cases. Facial pain, nasal obstruction, and facial paresthesia were the most common symptoms. Surgical resection (45.6%) was favored over biopsy (12.2%). Employing the free flap technique (4.7%), surgical reconstruction of the bony defect after resection was performed using abdominal and anterior thigh muscle grafts in 1.8% of patients each. As adjuvant management, 22.8% of cases had radiotherapy and 14.6% received chemotherapy. Recurrence of skull base ACCs occurred in 26.9% of cases during a mean follow up-time of 30.8±1.8 months.
Skull base ACCs pose a surgical challenge mainly due to their proximity to critical neurovascular structures and aggressive behavior. Surgical resection and radiotherapy are shown to be safe and effective treatment modalities. The dismal prognosis and limited data on non-surgical strategies highlight the need for further evaluation of the current management paradigm and upraising innovative therapies to improve patient mortality and quality of life.
背景/目的：系统评价侵犯颅底的腺样囊性癌（ACC）的患者特征及治疗方法。
根据PRISMA指南，检索PubMed、Scopus和Cochrane数据库，以获取报告颅底ACC患者治疗方案和生存结果的研究。对患者特征、治疗策略及结果进行调查。
该综述纳入17项研究，共171例患者，女性占优势（57.9%），平均年龄49±7.12岁。ACC最常侵犯鼻窦（22.2%）、海绵窦（8.8%）和鼻咽（7.1%）。6.4%的病例报告有神经侵犯。面部疼痛、鼻塞和面部感觉异常是最常见的症状。手术切除（45.2%）比活检（12.2%）更受青睐。采用游离皮瓣技术（4.7%），1.8%的患者在切除后使用腹部和股前肌移植进行骨缺损的手术重建。作为辅助治疗，22.8%的病例接受了放疗，14.6%接受了化疗。在平均30.8±1.8个月的随访期内，26.9%的颅底ACC病例出现复发。
颅底ACC主要因其靠近关键神经血管结构及侵袭性生长而给手术带来挑战。手术切除和放疗是安全有效的治疗方式。预后不佳以及非手术策略的数据有限，凸显了进一步评估当前治疗模式并开发创新疗法以提高患者生存率和生活质量的必要性。
