Clinicopathological Profile of Intraosseous Adenoid Cystic Carcinoma of the Jaws: A Systematic Review.

Objectives: We aimed to evaluate the clinicopathological features, survival rate, and potential prognostic markers of the jaws' primary intraosseous adenoid cystic carcinoma (PIACC).

Materials and methods: MEDLINE/PubMed, Scopus, and Embase searches were performed with the keywords "adenoid cystic carcinoma," and "jaw," or "maxilla," or "mandible." We included articles that evaluated cases diagnosed as PIACC in jaws. Studies with insufficient demographic data, inconclusive histopathologic diagnosis, and appropriate follow-up were excluded. The Joanna Briggs Institute tool was used to assess the risk of bias.

Results: Fifty-five PIACC comprising 27 studies met the inclusion criteria. The mean age of the patients was 56.4 ± 19.6 years with no sex predilection. PIACC showed a strong predilection for the mandible (69.1%), mainly in the posterior segment (40%). The patients presented symptoms in 87.3% of cases. Radiographically, PIACC presented as an ill-defined radiolucent lesion (40%). Most cases showed a cribriform pattern (32.7%). PIACC with a solid growth pattern presented a lower disease-free survival (DFS) (p = 0.040). The 2- and 5-year overall survival rates were 57.9% and 53.8%, respectively. Distant metastases were seen in 3.6% of the patients and were related to a lower DFS (p = 0.043).

Conclusion: PIACC is a rare neoplasm of the jaws with an incidence in the fifth and sixth decades of life and no sex predilection. The posterior mandible was affected in most cases. Solid growth patterns and distant metastases are prognostic factors for a lower DFS.