复发/转移性腺样囊性癌 (R/M AdCC) 提出了治疗选择有限的临床挑战，特别是在当前治疗方法的疗效不令人满意的情况下。本综述强调了管理 R/M AdCC 方面未得到满足的临床需求，并强调需要新的治疗策略来解决这一关键差距。本综述的主要目的是全面分析和评估研究 R/M AdCC 治疗方法的试验。重点放在肿瘤缓解率和无进展生存期等终点上。这些试验中检查的具体干预措施、人群和结果将得到详细说明，以提供有针对性和信息丰富的系统评价。系统检索跨越数据库，包括 PubMed、EMBASE 和 Cochrane 系统评价数据库。使用“癌、腺样囊性”和“试验”等术语，搜索重点关注 2010 年 4 月 1 日至 2023 年 8 月 9 日的英文全文文章。纳入标准包括针对 R/M AdCC 患者的研究，涉及药物干预。研究质量使用 Newcastle-Ottawa Scale 进行回顾性研究、Cochrane ROBINS-I 工具用于非随机试验、ROB-2 工具用于随机对照试验进行评估。本次评价共纳入了 46 项试验，涉及 1244 名患者，涵盖 R/M AdCC 的多种治疗方法。靶向治疗，特别是 500 mg 的阿帕替尼，具有 47.1% 的客观缓解率 (ORR) 的疗效。相反，免疫治疗药物表现不佳，总体 ORR 范围为 0 至 18%。虽然阿帕替尼显示出希望，但该综述强调了彻底探索针对 R/M AdCC 免疫冷性质的独特机制的药物的必要性。在早期临床试验的推动下，R/M AdCC 的全身治疗取得了明显进展。 ，特别是 VEGF-2 抑制剂的有希望的结果。然而，挑战仍然存在，特别是由于癌症的免疫冷性质而在免疫治疗方面。正在进行的研究（优先考虑早期试验）至关重要，强调对细胞疗法和抗体药物偶联物等新兴疗法的探索。过渡到 III 期试验对于获得更精确的治疗见解至关重要。协作努力和对个性化精准医疗的关注对于克服挑战和增进我们对这种罕见癌症治疗效果的理解至关重要。© 2024。作者，获得施普林格自然有限公司的独家许可。

Recurrent/metastatic adenoid cystic carcinoma (R/M AdCC) presents a clinical challenge with limited treatment options, particularly in the face of unsatisfactory efficacy from current therapeutic approaches. This review underscores the unmet clinical needs in managing R/M AdCC, emphasising the imperative for novel therapeutic strategies to address this critical gap.The primary objective of this review is to comprehensively analyse and assess trials investigating therapeutic approaches for R/M AdCC. Emphasis is placed on endpoints such as tumour response rates and progression-free survival. The specific interventions, populations, and outcomes examined in these trials will be detailed to provide a focused and informative systematic review.The systematic search spanned databases, including PubMed, EMBASE, and the Cochrane database of systematic reviews. Employing terms like "Carcinoma, Adenoid Cystic" and "trial," the search focused on English full-text articles from April 1, 2010, to August 9, 2023. Inclusion criteria encompassed studies with patients having R/M AdCC, involving drug interventions. Study quality was assessed using the Newcastle-Ottawa Scale for retrospective studies, Cochrane ROBINS-I tool for non-randomised trials, and the ROB-2 tool for randomised controlled trials.A total of 46 trials involving 1244 patients are included in this review, encompassing a variety of therapeutic approaches for R/M AdCC. Targeted therapies, particularly Apatinib at 500 mg, exhibit efficacy with a 47.1% objective response rate (ORR). Conversely, immunotherapeutic agents demonstrate suboptimal performance, with an overall ORR ranging from 0 to 18%. While Apatinib shows promise, the review underscores the imperative for a thorough exploration of drugs targeting unique mechanisms in the immunologically cold nature of R/M AdCC.Substantial progress in systemic therapy for R/M AdCC is evident, driven by early-phase clinical trials, particularly with promising outcomes in VEGF-2 inhibitors. However, challenges persist, notably in immunotherapy due to the cancer's immunologically cold nature. Ongoing research, prioritising early-stage trials, is crucial, emphasising exploration of emerging therapies like cell therapy and antibody-drug conjugates. Transitioning to Phase III trials is essential for more precise therapeutic insights. Collaborative efforts and a focus on personalised precision medicine are vital for overcoming challenges and advancing our understanding of treatment efficacy in this rare cancer.© 2024. The Author(s), under exclusive licence to Springer Nature Limited.