Hadj Saïd M, Ordioni U, Benat G, Gomez-Brouchet A, Chossegros C, Catherine J-H
Department of Oral Surgery, Odontology, Timone Hospital, 13385 Marseille, France; Department of Stomatology, Oral & Maxillofacial surgery, Conception Hospital, 13005 Marseille, France; UMR 7268 ADES, Faculty of Medicine, Aix-Marseille University/EFS/CNRS, 13344 Marseille, France.
Department of Oral Surgery, Odontology, Timone Hospital, 13385 Marseille, France.
J Stomatol Oral Maxillofac Surg. 2017 Dec;118(6):363-370. doi: 10.1016/j.jormas.2017.08.005. Epub 2017 Aug 30.
Clear cell odontogenic carcinoma (CCOC) is described as an exceptional and hard to diagnose malignant tumor which was first reported by Hansen in 1985. The purpose of this review article is to show that CCOC is a not that rare entity and to discuss its various aspects in order to enhance the diagnosis.
A search in the English language literature was performed using the Scopus, ScienceDirect, PubMed and Medline databases between 1985 and 2016. Data were collected on epidemiologic, clinical, radiographic, histological, immunohistochemistrical, cytogenetic, management, follow-up and prognosis features of CCOC.
Sixty-five studies from which a total of 95 case reports were included in the review. CCOC was generally seen in the fifth decade and the most common site was mandibular. The most frequently found symptoms were swelling, tooth mobility and pain. Radiologically, the image was radiolucent and could look like a cyst or a periodontal lesion. In situ hybridization techniques frequently expressed a gene fission of EWSR1. The treatment was mostly a radical surgical excision of the tumor with or without adjuvant radiotherapy or chemotherapy. CCOC showed high rates of recurrence and mortality related with the presence of distance metastasis.
Fission of EWSR1 gene could be the main element it the diagnosis of CCOC. A multidisciplinary approach, including a radiologist, pathologist and an oral & maxillofacial surgeon may be helpful in the evaluation and management of these lesions. With 95 reports found in English literature, we cannot say that CCOC is extremely rare anymore.
透明细胞牙源性癌（CCOC）是一种罕见且难以诊断的恶性肿瘤，于1985年由汉森首次报道。这篇综述文章的目的是表明CCOC并非那么罕见，并讨论其各个方面以提高诊断水平。
在1985年至2016年期间，使用Scopus、ScienceDirect、PubMed和Medline数据库对英文文献进行检索。收集了有关CCOC的流行病学、临床、影像学、组织学、免疫组织化学、细胞遗传学、治疗、随访和预后特征的数据。
65项研究被纳入综述，共95例病例报告。CCOC通常见于第五个十年，最常见的部位是下颌骨。最常见的症状是肿胀、牙齿松动和疼痛。在影像学上，图像呈透射性，可能类似囊肿或牙周病变。原位杂交技术经常显示EWSR1基因裂变。治疗主要是对肿瘤进行根治性手术切除，可辅以放疗或化疗。CCOC的复发率和死亡率较高，与远处转移的存在有关。
EWSR1基因裂变可能是CCOC诊断的主要因素。多学科方法，包括放射科医生、病理科医生和口腔颌面外科医生，可能有助于这些病变的评估和管理。在英文文献中发现了95篇报告，我们不能再说CCOC极其罕见了。
