EWSR1 and ATF1 rearrangements in clear cell odontogenic carcinoma: presentation of a case

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

“…Clear cell changes have been observed in a number of odontogenic lesions. [2][3][4][5][6][7][8][9]66,77 This is not surprising, because such changes have been described in residual odontogenic epithelium (rests of Serres). 77 Changes in clear cells have been attributed to many factors, including intracellular accumulation of nonstaining compounds such as glycogen, water, and mucin; a scarcity of cell organelles; and artifacts induced during tissue fixation or processing.…”

“…From the publications obtained in this search, those pertaining to cases of CCOC were recovered and included in the analysis. [2][3][4][5][6][7][8][9]39, Additionally, a manual search was conducted by cross-referencing the retrieved manuscripts. 38,40,[72][73][74][75][76] Subsequently, the studies reporting inconsistent histologic diagnosis or those from which the clinicopathologic data retrieval was difficult were excluded.…”

“…At that time, Dr. Mervin Shear defined this lesion as CCOC. 2,3 Four years later, Waldron, Small, and Silverman (1985) 4 described another 2 cases, which they called clear cell ameloblastoma (AME); 1 of these patients was diagnosed with regional metastasis. In the same year, Hansen et al (1985) 5 Additional publications in the late 1980s and early 1990s described similar lesions with aggressive behavior and metastatic potential under various nomenclatures, including clear cell AME, AME with clear cell differentiation, CCOT ameloblastic carcinoma (AMECA) with clear cell component, and CCOC.…”

Clear Cell Odontogenic Carcinoma (CCOC): Mini-Review of Literature and Case Report of Mandibular Radiolucency in 17-year Girl

“…Recent molecular studies have reported that both HCCC and CCOC have EWSR1-ATF1 translocation, and 83% of CCOC had EWSR1 rearrangement [14]. Balanced reciprocal translocations has been observed in approximately one-third of the sarcomas and EWSR1-ATF1 translocation in CCOC could signal the aggressiveness of this lesion [14]. Primary intraosseous localization, absence of any salivary gland tissue, and desmoplastic ameloblastoma-like areas helped differentiate our case from HCCC.…”

“…The location and the primary site of the lesion is the main diagnostic criterion to distinguish the two [4,10]. Recent molecular studies have reported that both HCCC and CCOC have EWSR1-ATF1 translocation, and 83% of CCOC had EWSR1 rearrangement [14]. Balanced reciprocal translocations has been observed in approximately one-third of the sarcomas and EWSR1-ATF1 translocation in CCOC could signal the aggressiveness of this lesion [14].…”