Study on clinical and biological characteristics of ameloblastic carcinoma
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RESEARCH
Study on clinical and biological characteristics of ameloblastic carcinoma Zhixing Niu1,2†, Ye Li3†, Wantao Chen4, Junfang Zhao1, Hongyu Zheng1, Qing Deng5, Zhian Zha1, Hao Zhu2,3, Qiang Sun1* and Lei Su6*
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 Background Ameloblastoma is a common odontogenic epithelial tumor that can transform into a malignant tumor called ameloblastic carcinoma (AC), which is very rare [1]. In the latest edition of the 2017 World Health Organization (WHO) classification of odontogenic tumors, AC was defined as a rare odontogenic malignancy that combines the histologic features of ameloblastoma with cytologic atypia, having a 5-year survival rate of 69.1% [2, 3]. AC occurs mainly in the posterior mandible and presents as two main types: a primary type called de novo cancer and a secondary type, defined as a malignant transformation from a pre-existing benign ameloblastoma [3, 4].
*Correspondence: [email protected]; [email protected] † Zhixing Niu and Ye Li contributed equally to this work 1 Department of Stomatology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, Zhengzhou 450052, Henan Province, China 6 Department of Radiology, The First Affiliated Hospital of Zhengzhou University, No. 1, East Jian she Road, Zhengzhou, Henan Province, China Full list of author information is available at the end of the article
Unfortunately, AC has a high recurrence rate after surgery, causing invasive and extensive bone destruction; its clinical diagnosis and treatment are very challenging. Transformation may be closely associated with a long medical history, multiple operations, radiotherapy, and chemotherapy, but the mechanisms of malignant transformation are poorly understood. Therefore, early tumor diagnosis and treatment are crucial. In recent years, with the rapid development of molecular biology, some studies also reported a BRAF-V600E mutation rate of approximately 60% in ameloblastoma [5–7]. The BRAF gene is an important proto-oncogene that plays an important role in tumor cell proliferation, differentiation, and apoptosis. Therefore, the presence of BRAF-V600
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