■小唾液腺腺样囊性癌(AdCCmSG)是罕见的,\'在缺乏标准化方面构成了临床挑战,基于证据的建议。目前,AdCCmSG管理主要是从主要唾液腺癌(MSGC)翻译而来的。理想情况下,应在多学科范围内讨论和进行AdCCmSG诊断-治疗检查,高专业知识设置,包括病理学家,外科医生,放射肿瘤学家和医学肿瘤学家。
■本综述概述了流行病学和病理分类。此外,最近的,AdCCmSG治疗的临床相关更新(Pubmed搜索,具体准则)进行了批判性讨论,为了更好地了解这种罕见的病理实体,有可能优化护理过程,并为反思未来的治疗发展提供了起点。
■罕见癌症的管理往往受到有限的数据和临床试验的阻碍,缺乏循证指南,几乎不代表疾病异质性,这不能用“一刀切”的方法成功解决。我们的目标是解决这些潜在的陷阱,提供一个易于使用的,已更新,关于AdCCmSG管理的多学科专家意见收集,作为今天的临床实践。我们还将涵盖最有希望的未来前景,基于AdCCmSG分子背景中突出显示的潜在治疗靶点。
UNASSIGNED: Adenoid cystic carcinoma of minor salivary glands (AdCCmSG) represents a \'rarity in the rarity,\' posing a clinical challenge in lack of standardized, evidence-based recommendations. At present, AdCCmSG management is mostly translated from major salivary gland cancers (MSGCs). Ideally, AdCCmSG diagnostic-therapeutic workup should be discussed and carried out within a multidisciplinary, high-expertise setting, including pathologists, surgeons, radiation oncologists and medical oncologists.
UNASSIGNED: The present review provides an overview of epidemiology and pathologic classification. Moreover, the most recent, clinically relevant updates in the treatment of AdCCmSG (Pubmed searches, specific guidelines) are critically discussed, aiming to a better understanding of this rare pathologic entity, potentially optimizing the care process, and offering a starting point for reflection on future therapeutic developments.
UNASSIGNED: The management of rare cancers is often hindered by limited data and clinical trials, lack of evidence-based guidelines, and hardly represented disease heterogeneity, which cannot be successfully tackled with a \'one-size-fits-all\' approach. Our goal is to address these potential pitfalls, providing an easy-to-use, updated, multidisciplinary collection of expert opinions concerning AdCCmSG management as of today\'s clinical practice. We will also cover the most promising future perspectives, based on the potential therapeutic targets highlighted within AdCCmSG\'s molecular background.