Clear cell odontogenic carcinoma: report of 6 cases and review of the literature

Abstract

Clear cell odontogenic carcinoma (CCOC) is a rare malignant tumor. To date, only 67 cases were described in the English literature and complete understanding of the behavior of CCOC was based on limited case reports. In this article, we reported 6 additional cases and reviewed the relevant literature. Our cases included 4 men and 2 women with an average age of 52.8 years. The tumors were all in the mandible and presented as poorly marginated radiolucencies. Patients were treated with resection. All cases consisted of islands and sheets of clear cells that were separated by fibrous septa. Immunohistochemically, the tumor cells were positive for pan-cytokeratins, CK14, CK19, and epithelial membrane antigen, but negative for S-100 protein, smooth muscle actin, desmin, human melanoma antigen, CD3, CD45, and glial fibrillary acidic protein. A series of literature review disclosed the tumor has a female preponderance (44 female vs. 23 male, 1.9:1). The mean age was 54.5 years and the predominant site was the mandible (51, 76.1%). Forty-seven (70.1%) patients were initially treated with surgical resection, 15 (22.4%) patients underwent curettage or enucleation, and 1 patient was treated with chemotherapy. Nine patients had a neck dissection in addition to a surgical resection. Seven (9.6%) patients received postoperative radiotherapy. The recurrence rate after resection was lower than conservative therapy (29 vs. 86.7%). Nine patients died of the tumor. CCOC is a potentially aggressive lesion and owing to a high recurrent rate of conservative measure; therefore, radical surgical treatment is recommended and long-term follow-up is necessary.

Clear cell odontogenic carcinoma: report of 7 new cases and systematic review of the current knowledge

“…On the basis of our and others' findings, most cases of CCOC affect the posterior mandible of women during the sixth decade of life, with no ethnic predilection. In contrast to previous reports in other reviews, 40,43,49,52,55 anterior mandible regions (24, 25.5%) appear to bear lower numbers of tumors compared with the posterior region (40, 42.6%).…”

“…CKs 7, 8, and 18 were absent or mildly expressed, similar to findings in other investigations. 18,19,25,27,35,40,47,52,55 Other antigens have been reported as negative (vimentin, desmin, enolase, smooth muscle actin, calponin, S-100 protein, human melanoma black-45, a(1)-chymotrypsin, CD10, CD31, CD45, glial fibrillary acidic protein, and chromogranin) or mildly positive in expression (CK13 and EMA). 8,18,19,26,27,31,40,47 The previously metioned immunohistochemical markers can be useful in the differential diagnosis of epithelial-myoepithelial salivary gland tumors, melanocytic lesions, and lymphatic tumors.…”

Malignant Odontogenic Tumors: An Update on Selected Tumors

“…Hansen originally described it in 1985 as a benign but aggressive lesion, centrally located in the jaws, under the guise of clear cell odontogenic tumor [20]. Subsequent case reports and small series included additional clinical follow-up that documented the capacity of this neoplasm for local destructive growth with invasion of medullary bone, nerves, lymphatics, as well as regional lymph node and distant metastases (pulmonary, bone) [21][22][23]. The World Health Organization reclassified the tumor in 2005 as an odontogenic carcinoma ( Table 1).…”

“…Long-term follow up is necessary for these patients since metastases can occur years later after primary resection. Patients who died of the tumor developed distant metastases [23].…”

Management of Clear Cell Odontogenic Carcinoma: A Case Report

“…Such tumors include calcifying epithelial odontogenic tumor and clear cell ameloblastoma. While the former is identified by the presence of psammomatous calcifications and amyloid deposits, the latter may be difficult to distinguish from CCOC [2]. In CCOC, surgical resection with a wide margin is the treatment of choice.…”