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.
Highlights
Ameloblastic carcinoma (AC) is an odontogenic malignant tumor which is closely related to benign ameloblastoma
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]
Summary
Ameloblastic carcinoma (AC) is an odontogenic malignant tumor which is closely related to benign ameloblastoma. 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]. AC has a high recurrence rate after surgery, causing invasive and extensive bone destruction; its clinical diagnosis and treatment are very challenging.
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