Yarbrough Wendell G, Panaccione Alexander, Chang Michael T, Ivanov Sergey V
Section of Otolaryngology, Department of Surgery, Yale School of Medicine New Haven Connecticut USA.
Yale Cancer Center New Haven Connecticut USA.
Laryngoscope Investig Otolaryngol. 2016 Aug 4;1(4):60-77. doi: 10.1002/lio2.22. eCollection 2016 Aug.
This review surveys trialed therapies and molecular defects in adenoid cystic carcinoma (ACC), with an emphasis on neural crest-like stemness characteristics of newly discovered cancer stem cells (CSCs) and therapies that may target these CSCs.
Articles available on Pubmed or OVID MEDLINE databases and unpublished data.
Systematic review of articles pertaining to ACC and neural crest-like stem cells.
Adenoid cystic carcinoma of the salivary gland is a slowly growing but relentless cancer that is prone to nerve invasion and metastases. A lack of understanding of molecular etiology and absence of targetable drivers has limited therapy for patients with ACC to surgery and radiation. Currently, no curative treatments are available for patients with metastatic disease, which highlights the need for effective new therapies. Research in this area has been inhibited by the lack of validated cell lines and a paucity of clinically useful markers. The ACC research environment has recently improved, thanks to the introduction of novel tools, technologies, approaches, and models. Improved understanding of ACC suggests that neural crest-like stemness is a major target in this rare tumor. New cell culture techniques and patient-derived xenografts provide tools for preclinical testing.
Preclinical research has not identified effective targets in ACC, as confirmed by the large number of failed clinical trials. New molecular data suggest that drivers of neural crest-like stemness may be required for maintenance of ACC; as such, CSCs are a target for therapy of ACC.
本综述调查腺样囊性癌（ACC）的试验性疗法和分子缺陷，重点关注新发现的癌症干细胞（CSC）的神经嵴样干性特征以及可能靶向这些CSC的疗法。
可在PubMed或OVID MEDLINE数据库上获取的文章以及未发表的数据。
对与ACC和神经嵴样干细胞相关的文章进行系统综述。
涎腺腺样囊性癌是一种生长缓慢但侵袭性强的癌症，易于侵犯神经并发生转移。对分子病因的了解不足以及缺乏可靶向的驱动因子，使得ACC患者的治疗仅限于手术和放疗。目前，转移性疾病患者尚无治愈性治疗方法，这凸显了有效新疗法的必要性。该领域的研究因缺乏经过验证的细胞系和临床可用标志物的匮乏而受到抑制。由于引入了新的工具、技术、方法和模型，ACC的研究环境最近有所改善。对ACC的进一步了解表明，神经嵴样干性是这种罕见肿瘤的主要靶点。新的细胞培养技术和患者来源的异种移植为临床前测试提供了工具。
临床前研究尚未在ACC中确定有效靶点，大量失败的临床试验证实了这一点。新的分子数据表明，神经嵴样干性驱动因子可能是维持ACC所必需的；因此，CSC是ACC治疗的一个靶点。
