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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2020

Open Access 01-12-2020 | Targeted Therapy | Research

Study on clinical and biological characteristics of ameloblastic carcinoma

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

Published in: Orphanet Journal of Rare Diseases | Issue 1/2020

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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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Metadata
Title
Study on clinical and biological characteristics of ameloblastic carcinoma
Authors
Zhixing Niu
Ye Li
Wantao Chen
Junfang Zhao
Hongyu Zheng
Qing Deng
Zhian Zha
Hao Zhu
Qiang Sun
Lei Su
Publication date
01-12-2020
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2020
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-020-01603-5

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