Study on clinical and biological characteristics of ameloblastic carcinoma

Orphanet J Rare Dis. 2020 Nov 11;15(1):316. doi: 10.1186/s13023-020-01603-5.

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.

Keywords: Ameloblastic carcinoma; Ameloblastoma; BRAF gene; Targeted therapy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ameloblastoma* / genetics
  • Ameloblastoma* / surgery
  • Carcinoma*
  • Humans
  • Middle Aged
  • Odontogenic Tumors*