Department of Nasopharyngeal Carcinoma, Sun Yat-Sen University Cancer Center, 651 Dongfeng East Road, Guangzhou, Guangdong, 510060, P.R. China.
Sun Yat-Sen University Cancer Center; State Key Laboratory of Oncology in South China; Collaborative Innovation Center for Cancer Medicine; Guangdong Key Laboratory of Nasopharyngeal Carcinoma Diagnosis and Therapy, Guangzhou, 510060, China.
BMC Cancer. 2024 May 10;24(1):576. doi: 10.1186/s12885-024-12159-z.
Nasopharyngeal adenoid cystic carcinoma (NACC) is a rare malignancy with special biological features. Controversies exist regarding the treatment approach and prognostic factors in the IMRT era. This study aimed to evaluate the long-term outcomes and management approaches in NACC.
Fifty patients with NACC at our institution between 2010 and 2020 were reviewed. Sixteen patients received primary radiotherapy (RT), and 34 patients underwent primary surgery.
Between January 2010 and October 2020, a total of 50 patients with pathologically proven NACC were included in our analysis. The median follow-up time was 58.5 months (range: 6.0-151.0 months). The 5-year overall survival rate (OS) and progression-free survival rate (PFS) were 83.9% and 67.5%, respectively. The 5-year OS rates of patients whose primary treatment was surgery and RT were 90.0% and 67.3%, respectively (log-rank P = 0.028). The 5-year PFS rates of patients whose primary treatment was surgery or RT were 80.8% and 40.7%, respectively (log-rank P = 0.024). Multivariate analyses showed that nerve invasion and the pattern of primary treatment were independent factors associated with PFS.
Due to the relative insensitivity to radiation, primary surgery seemed to provide a better chance of disease control and improved survival in NACC. Meanwhile, postoperative radiotherapy should be performed for advanced stage or residual tumours. Cranial nerve invasion and treatment pattern might be important factors affecting the prognosis of patients with NACC.
鼻咽腺样囊性癌（NACC）是一种具有特殊生物学特征的罕见恶性肿瘤。在调强放疗（IMRT）时代，其治疗方法和预后因素仍存在争议。本研究旨在评估 NACC 的长期疗效和治疗方法。
回顾性分析 2010 年至 2020 年间我院收治的 50 例 NACC 患者的临床资料。16 例患者接受了单纯放疗（RT），34 例患者接受了手术治疗。
2010 年 1 月至 2020 年 10 月，共纳入 50 例病理证实的 NACC 患者，中位随访时间为 58.5 个月（范围：6.0-151.0 个月）。患者的 5 年总生存率（OS）和无进展生存率（PFS）分别为 83.9%和 67.5%。单纯手术和单纯 RT 治疗患者的 5 年 OS 率分别为 90.0%和 67.3%（log-rank P=0.028）。单纯手术和单纯 RT 治疗患者的 5 年 PFS 率分别为 80.8%和 40.7%（log-rank P=0.024）。多因素分析显示，神经侵犯和初始治疗方式是影响 PFS 的独立因素。
由于对放疗相对不敏感，原发肿瘤手术似乎能更好地控制疾病并改善 NACC 患者的生存。同时，对于晚期或残留肿瘤应行术后放疗。颅神经侵犯和治疗方式可能是影响 NACC 患者预后的重要因素。
