Abu-Taleb, NSAbou-Alnour, DAFarag, AF2019-12-222019-12-222018-122233-7822https://doi.org/10.5624/isd.2018.48.4.233https://www.ncbi.nlm.nih.gov/pubmed/30607347Accession Number: WOS:000454326600001Purpose: Oral carcinoma cuniculatum is a rare well-differentiated variant of oral squamous cell carcinoma. The purpose was to systematically review its unique features to differentiate it from other variants as verrucous carcinoma, papillary squamous cell carcinoma and well-differentiated squamous cell carcinoma. Materials and Methods: A systematic review was performed using MEDLINE, Dentistry and Oral Sciences Source and PubMed databases and any existing articles related to the research subject missed in the search strategy to screen ones reporting cases occurring exclusively in the oral cavity in English literature. Variables analyzed included clinical, etiologic, imaging, histopatholgical features, treatment, follow-up and survival rates. Results: From 229 hits, 17 articles with 43 cases were included in the systematic review. Clinically it showed a female predilection with pain and/or ulceration of a relatively long duration and exudation being the most common symptoms. Histologically, it showed more endophytic features comprising well-differentiated squamous epithelium with absent or minimal cytological atypia and multiple keratin filled crypts or cuniculus. Inflammatory stromal reaction and discharging abscesses were reported in most of the cases. Bone destruction was predominant in most imaging features. Complete surgical resection with a safety margin was the treatment of choice in most of the cases with few recorded recurrence cases. Conclusion: Apprehensive knowledge of oral carcinoma cuniculatum unique features is essential to avoid its misdiagnosis and provide proper treatment especially for recurrent casesen-USUniversity for ENTITYDIAGNOSISVerrucousCarcinomaSquamous CellCarcinomaMoutHArticlehttps://doi.org/10.5624/isd.2018.48.4.233