Study on clinical and biological characteristics of ameloblastic carcinoma

Orphanet Journal of Rare Diseases

Hao Zhu¹  Wantao Chen²  Qing Deng³  Ye Li⁴  Lei Su⁵  Qiang Sun⁶  Hongyu Zheng⁶  Zhian Zha⁶  Junfang Zhao⁶  Zhixing Niu⁷ 

[1] Academy of Medical Sciences, Zhengzhou University, No. 100 Science Avenue, 450000, Zhengzhou, Henan Province, China;Department of Pathology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, 450052, Zhengzhou, Henan Province, China;Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, 200011, Shanghai, China;Department of Pathology, The First Affiliated Hospital of Henan University of Science and Technology, No. 24 Jinghua Road, Jianxi District, 471003, Luoyang City, Henan Province, China;Department of Pathology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, 450052, Zhengzhou, Henan Province, China;Department of Radiology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, Zhengzhou, Henan Province, China;Department of Stomatology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, 450052, Zhengzhou, Henan Province, China;Department of Stomatology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, 450052, Zhengzhou, Henan Province, China;Academy of Medical Sciences, Zhengzhou University, No. 100 Science Avenue, 450000, Zhengzhou, Henan Province, China;

关键词: Ameloblastic carcinoma;    Ameloblastoma;    BRAF;    Targeted therapy;   

DOI  :  10.1186/s13023-020-01603-5

来源: Springer

【 摘 要 】

BackgroundAmeloblastic carcinoma (AC) is an odontogenic malignant tumor which is closely related to benign ameloblastoma. Because of its rarity, diagnosis and treatment are difficult. In this study, we summarized and analyzed the clinical and biological characteristics of AC.ResultsFifteen patients with AC and a median age of 53 years were identified. Among of them, five patients who were tested carried a BRAF-V600E mutation. Two patients presented with cervical lymph nodes and lung metastases. Primary AC was more invasive, and the bone destruction ability of the primary type was more radical than that of the secondary type.ConclusionsThis study revealed that the BRAF-V600E mutation was related to the aggressive behavior of AC, and early radical resection is crucial. Moreover, targeted therapy may be a new direction in the future.

【 授权许可】

CC BY