成釉细胞瘤是良性的,需要广泛手术切除的局部侵袭性肿瘤。本文的目的是对良性成釉细胞瘤进行深入的综述,以确定可用的证据水平以及靶向治疗成釉细胞瘤和BRAFV600E突变在成釉细胞瘤中的治疗的可能益处。根据PubMed/MEDLINE中的PRISMA指南进行了电子文献检索,EBSCO,和WebofScience在1975年至2021年之间发表的合格研究。系统评价在INPLASY(INPLASY202260018)注册。审查包括2例病例系列和17例病例报告。组织病理学类型,解剖位置,BRAF突变的表达,额外的突变,和分子靶向治疗的19篇综述的文章进行了总结和列表。有趣的是,成釉细胞瘤的大部分原发部位位于下颌骨(80.9%),而上颌骨(17%).在9项纳入的研究中报告了肿瘤大小。该综述中大多数纳入的研究显示具有BRAFV600E突变的成釉细胞瘤,并对分子靶向治疗有反应。基于有限的现有证据,在具有BRAFV600E突变的成釉细胞瘤中使用BRAF和/或MEK抑制剂的分子疗法被证明是适当的治疗方法。必须进一步加深我们在临床和分子水平上的理解,以提高成釉细胞瘤的治疗精度。
Ameloblastoma is a benign, locally aggressive neoplasm that needs extensive surgical resection. The goal of this article is to obtain an in-depth review of benign ameloblastomas to determine the available level of evidence and the possible benefit of targeted therapeutics for the treatment of ameloblastoma and BRAF V600E mutation in ameloblastoma. An electronic literature search was conducted according to PRISMA guidelines in PubMed/MEDLINE, EBSCO, and Web of Science for eligible studies published between 1975 and 2021. The systematic review is registered with INPLASY (INPLASY202260018). The review included 2 case series and 17 case reports. The histopathological type, anatomic location, expression of BRAF mutation, additional mutations, and molecular-targeted therapies of the 19 reviewed articles were summarized and tabulated. Interestingly, the majority of the primary site of ameloblastoma was located in the mandible (80.9%) compared to the maxilla (17%). The tumour size was reported in nine of the included studies. Most of the included studies in the review exhibited ameloblastoma with BRAF V600E mutations and responded to molecular-targeted therapies. Molecular therapies employing BRAF and/or MEK inhibitors in ameloblastoma with BRAF V600E mutations proved to be an appropriate treatment based on the limited available evidence. It is essential further to deepen our understanding at the clinical and molecular level to enhance the precision of management of ameloblastoma.