背景:由小涎腺(MSG)引起的口腔内腺样囊性癌(ACC)是一种罕见的恶性肿瘤,与诊断延迟和不良预后相关。本研究旨在全面回顾MSG的ACC,专注于临床特征,成像模式,治疗方法,和长期结果。
方法:在PubMed中进行了系统搜索,WebofScience,和MEDLINE数据库,以确定报告1997年1月至2023年3月期间MSGACC案例的相关文章。该研究在PROSPERO注册(ID:CRD42023449478)。总共选择了10项符合纳入标准的研究进行关键审查。总的来说,902例患者诊断为ACCMSG,年龄范围为44.3至63岁,平均年龄为56.6岁。女性与男性的比例范围从1:1到2.4:1。关于ACC的主要地点,腭是最常见的位置,占30.5%至83.3%,其次是颊粘膜,嘴巴的地板,嘴唇和后磨牙区。对于组织学,固体质量模式是最普遍的,在95.2%的患者中看到,其次是卷曲图案。关于治疗方式,手术是最常见的方法,在76.3%的病例中应用,29.0%的病例采用手术和放疗相结合。一小部分,3.2%,接受了联合手术,化疗,和放射治疗,8.3%仅接受放疗。局部复发率在1%到28.5%之间,远处转移发生在18.2%至33.3%的病例中,主要到淋巴结(14.5%)。对不同阶段和患者数量的总体生存率的分析表明,5年生存率为68.0%。这项研究的结果为医生制定治疗决策提供了有价值的见解,并强调需要进行持续的研究和临床合作努力来改善这种具有挑战性的疾病的管理和结果。
结论:MSG的ACC是一种多面性疾病,通常表现为无症状性增大和溃疡。这种疾病的特点是不同的组织病理学模式和神经周浸润(PNI)。认识到这些独特的方面是制定治疗计划的关键,从外科手术到放射治疗,化疗,和不断发展的靶向治疗。持续的研究和协作的临床努力对于这种具有挑战性的疾病的治疗和管理的持续进展仍然至关重要。
BACKGROUND: Intraoral adenoid cystic carcinoma (ACC) arising from minor salivary glands (MSG) is a rare malignancy associated with delayed diagnosis and unfavorable outcomes. This study aimed to comprehensively review ACC of MSGs, focusing on clinical characteristics, imaging modalities, treatment approaches, and long-term outcomes.
METHODS: A systematic search was conducted in PubMed, Web of Science, and MEDLINE databases to identify relevant articles reporting cases of ACC of MSGs between January 1997 and March 2023. The study was registered in PROSPERO (ID: CRD42023449478). A total of 10 studies that met the inclusion criteria were selected for critical review. In total, 902 patients were diagnosed with ACC of MSGs with an age range of 44.3 to 63 years, and an average age of 56.6 years. The female to male ratio ranges from 1:1 to 2.4:1. Regarding the primary site of ACC, the palate was the most common location, accounting for 30.5% to 83.3%, followed by the buccal mucosa, floor of the mouth, and lip and the retromolar area. For histology, the solid mass pattern was the most prevalent, seen in 95.2% of patients, followed by the cribriform pattern. Regarding treatment modalities, surgery was the most common approach, applied in 76.3% of cases, with a combination of surgery and radiotherapy used in 29.0% of cases. A smaller fraction, 3.2%, received a combination of surgery, chemotherapy, and radiotherapy, and 8.3% underwent radiotherapy alone. Local recurrence rates varied between 1% and 28.5%, and distant metastasis occurred in 18.2% to 33.3% of cases, predominantly to lymph nodes (14.5%). An analysis of overall survival across various stages and patient numbers indicated a 5-year survival rate of 68.0%. The findings of this study provide valuable insights for physicians in making treatment decisions and emphasize the need for ongoing research and collaborative clinical efforts to improve the management and outcomes of this challenging disease.
CONCLUSIONS: ACC of MSGs is a multifaceted condition typically manifesting as asymptomatic enlargement and ulceration. This disease is marked by distinct histopathological patterns and perineural invasion (PNI). Recognizing these distinctive aspects is key in shaping the treatment plan, which can range from surgical procedures to radiation therapy, chemotherapy, and evolving targeted treatments. Continuous research and collaborative clinical efforts remain critical for ongoing progress in the treatment and management of this challenging condition.