从分子生物学角度对成釉细胞瘤诊断及治疗的考量  

Diagnosis and treatment of ameloblastoma from molecular biology perspective

作　　者：钱颖 龚佳幸 俞梦飞[1] 刘宇 魏栋[2] 朱子羽 陆科杰 王慧明[1] Qian Ying;Gong Jiaxing;Yu Meng‐fei;Liu Yu;Wei Dong;Zhu Ziyu;Lu Kejie;Wang Huiming(Dept.of Oral Implantology,The Affiliated Hospital of Stomatology,School of Stomatology,Zhejiang University School of Medicine,Key Laboratory of Oral Biomedical Re‐search of Zhejiang Province,Hangzhou 310006,China;Dept.of Oral and Maxillofacial Surgery,The First Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310006,China)

机构地区：[1]浙江大学医学院附属口腔医院口腔种植中心,浙江大学口腔医学院,浙江省口腔生物医学研究重点实验室,杭州310006 [2]浙江大学医学院附属第一医院口腔颌面外科,杭州310006

出　　处：《国际口腔医学杂志》2021年第5期570-578,共9页International Journal of Stomatology

基　　金：国家重点研究发展计划(2018YFA0703000);国家自然科学基金(81670972);浙江省重点研究发展计划(2017C01054,2018-C03062,2017C01063);中国博士后科学基金(2020TQ0257,2020M-681896)。

摘　　要：成釉细胞瘤是较为常见的牙源性肿瘤。它易复发、易恶变,属于临界瘤。成釉细胞瘤具有许多特殊的标志物,虽然目前其组织来源及发病机制尚未明确,但其发生与多种信号转导途径密切相关,包括有丝分裂原活化蛋白激酶(MAPK)信号通路、Sonic Hedgehog(SHH)信号通路和经典WNT/β-catenin信号通路。鼠类肉瘤滤过性毒菌致癌同源体B1(BRAF)突变、大鼠肉瘤病毒致癌基因同源物(RAS)突变和Smoothened(SMO)突变等多种突变基因型已在成釉细胞瘤中被发现,不同突变型的成釉细胞瘤有不同的临床特点和生物学行为,结合组织学特点可用于指导分型,对治疗及预后具有重要意义。为了进一步探讨这种与临床相关的基因型-表型相关性,本文就成釉细胞瘤的分类、分子水平标志物、发病机制、靶向治疗及预后进行综述,以期对今后的诊断和治疗有所帮助。Ameloblastoma is the most common odontogenic tumor. It is prone to relapse and malignant transformation,and it should be a borderline tumor. To date, several special markers for ameloblastoma have been found. Although the tissue source and pathogenesis of ameloblastoma are not entirely clear, numerous signal transduction pathways have been found closely associated with its occurrence, such as the mitogen-activated protein kinase signaling pathway, Sonic Hedgehog signaling pathway, and canonical WNT/β-catenin signaling pathway. Recently, multiple mutant genes have been found in ameloblastoma, including Vraf murine sarcoma viral oncogene homolog B1(BRAF)mutations, rat sarcoma viral oncogene homolog(RAS)mutations, and smoothened(SMO) mutations. Different mutant ameloblastomas have different clinical characteristics and biological behavior. The histological characteristics of ameloblastomas can guide typing, and they have distinguished significance for treatment and prognosis. This review will focus on classification, molecular level markers, pathogenesis, targeted therapy, and prognosis of ameloblastoma to explore clinically relevant genotype-phenotype correlations and assist future diagnosis and treatment.

关 键 词：成釉细胞瘤 基因突变 有丝分裂原活化蛋白激酶信号通路 Sonic Hedgehog信号通路 靶向治疗开 

分 类 号：R739.81[医药卫生—肿瘤]