RESUMO
Clear cell Odontogenic Carcinoma (CCOC) is an uncommon malignant odontogenic tumor (MOT). It is the fifth most common MOT. A systematic review is presented of reported cases, case series and retrospective studies of CCOC, to determine trends in presentation, diagnostic features, treatment, and patient outcome. Searches of detailed databases were carried out to identify papers reporting CCOC. The variables were demographics, patient symptoms, tumor location, histopathological findings, immunohistochemical studies, treatment, follow-up, and recurrence. 117 cases were identified; CCOC was most frequently seen in mature females 65% (n = 76). The total average age was 55.4 with a range from 17 to 89 years, for females 56.4 and males 53.6 years. The mean size was 3.41 cm. The most common location was in the mandibular body 36.2% (n = 42), followed by the anterior mandible 23.3% (n = 27). The most common clinical presentation was a swelling 80.4% (n = 74), and the main symptom was pain 41.3% (n = 31), followed by painless lesion 24% (n = 18). The most common Immunohistochemistry positive expression was CK19, EMA, and CEA, and for special staining periodic acid Shiff (PAS); 97% of cases were treated surgically. The average follow-up was 30.3 months, and recurrence was reported in 52.4% of the cases. Conclusion: CCOC shows a strong predilection for the body and anterior mandible, and females are more frequently affected. CCOCs can be painful and the principle clinical sign is swelling, CCOCs can metastasize, and the prognosis is fair.