Primary intraosseous adenoid cystic carcinoma of the mandible: a comprehensive review and analysis of 4 new cases with emphasis on morphologic, immunophenotypic and molecular characteristics

Publication date: Available online 11 November 2016
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Jing Han, Ting Gu, Xi Yang, Longwei Hu, Ronghui Xia, Zhen Tian, Jiang Li, Ling Zhu, Liqun Xu, Chunye Zhang
ObjectiveTo comprehensively review the clinical manifestations, imaging, diagnosis, treatment and pathological features of primary intraosseous adenoid cystic carcinoma (PIACC) of the mandible as well as to analyze PIACC histopathology and molecular features in 4 patient cases.Study DesignWe reviewed the literature and retrospectively studied 4 patient cases of PIACC.ResultsThe clinical and imaging findings of PIACC are similar to other malignant or borderline-malignant mandible tumors. The 4 cases of PIACC included 3 males and 1 female between 41 and 58 years of age. The histopathological features of the tumors resembled those of ACC. We observed abundant osteoclasts resorbing bone at the leading edge of the tumors characterized by solid structure histology but not by the cribriform subtype. Additionally, all 4 cases showed abnormalities in the MYB gene and high expression of MYB protein. All patients survived for the duration of follow-up, and 2 patients showed distant metastases (followed up 3-36 months).ConclusionsPIACC is extremely rare and is often clinically misdiagnosed. Different histological subtypes could show different mechanisms of invasion of the mandible. MYB gene and protein expression abnormalities can be used as indicators for the precise diagnosis of PIACC.



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