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Study on clinical and biological characteristics of ameloblastic carcinoma

Authors
  • Niu, Zhixing1, 2
  • Li, Ye1
  • Chen, Wantao3
  • Zhao, Junfang1
  • Zheng, Hongyu1
  • Deng, Qing4
  • Zha, Zhian1
  • Zhu, Hao2, 1
  • Sun, Qiang1
  • Su, Lei5
  • 1 The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, Zhengzhou, Henan Province, 450052, China , Zhengzhou (China)
  • 2 Zhengzhou University, No. 100 Science Avenue, Zhengzhou, Henan Province, 450000, China , Zhengzhou (China)
  • 3 Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, China , Shanghai (China)
  • 4 The First Affiliated Hospital of Henan University of Science and Technology, No. 24 Jinghua Road, Jianxi District, Luoyang City, Henan Province, 471003, China , Luoyang City (China)
  • 5 The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, Zhengzhou, Henan Province, China , Zhengzhou (China)
Type
Published Article
Journal
Orphanet Journal of Rare Diseases
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Nov 11, 2020
Volume
15
Issue
1
Identifiers
DOI: 10.1186/s13023-020-01603-5
Source
Springer Nature
Keywords
License
Green

Abstract

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.

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