Orphanet Journal of Rare Diseases (Nov 2020)

Study on clinical and biological characteristics of ameloblastic carcinoma

  • Zhixing Niu,
  • Ye Li,
  • Wantao Chen,
  • Junfang Zhao,
  • Hongyu Zheng,
  • Qing Deng,
  • Zhian Zha,
  • Hao Zhu,
  • Qiang Sun,
  • Lei Su

DOI
https://doi.org/10.1186/s13023-020-01603-5
Journal volume & issue
Vol. 15, no. 1
pp. 1 – 9

Abstract

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Abstract Background Ameloblastic 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. Results Fifteen 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. Conclusions This 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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