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Abstract：
Clear cell odontogenic carcinoma (CCOC) is a rare malignant odontogenic tumor (MOT) characterized by sheets and lobules of vacuolated and clear cells. To understand the biology of CCOC, we established a new cell line, CCOC-T, with EWSR1-ATF1 fusion gene from a mandible tumor with distant metastasis and characterized this cell line.
To detect the EWSR1-ATF1 fusion gene, we used three CCOC cases, including the present case, by RT-PCR and FISH analysis. We characterized established CCOC-T cells by checking cell growth, invasion and the expression of odontogenic factors and bone-related factors. Moreover, the gene expression profile of CCOC-T cells was examined by microarray analysis.
Histologically, the primary tumor was comprised of cords and nests containing clear and squamoid cells separated by fibrous septa. In addition, ameloblastomatous islands with palisaded peripheral cells were observed, indicating probable odontogenic origin. This tumor expressed the fusion gene EWSR1-ATF1, which underlies the etiology of hyalinizing clear cell carcinoma (HCCC) and potentially that of CCOC. We found a breakpoint in the EWSR1-ATF1 fusion to be the same as that reported in HCCC. Established CCOC-T cells grew extremely slowly, but the cells showed highly invasive activity. Moreover, CCOC-T cells expressed bone-related molecules, odontogenic factors, and epithelial mesenchymal transition (EMT)-related molecules.
To the best of our knowledge, this is the first report on the establishment of a CCOC cell line. CCOC-T cells serve as a useful in vitro model for understanding the pathogenesis and nature of MOT.
To detect the EWSR1-ATF1 fusion gene, we used three CCOC cases, including the present case, by RT-PCR and FISH analysis. We characterized established CCOC-T cells by checking cell growth, invasion and the expression of odontogenic factors and bone-related factors. Moreover, the gene expression profile of CCOC-T cells was examined by microarray analysis.
Histologically, the primary tumor was comprised of cords and nests containing clear and squamoid cells separated by fibrous septa. In addition, ameloblastomatous islands with palisaded peripheral cells were observed, indicating probable odontogenic origin. This tumor expressed the fusion gene EWSR1-ATF1, which underlies the etiology of hyalinizing clear cell carcinoma (HCCC) and potentially that of CCOC. We found a breakpoint in the EWSR1-ATF1 fusion to be the same as that reported in HCCC. Established CCOC-T cells grew extremely slowly, but the cells showed highly invasive activity. Moreover, CCOC-T cells expressed bone-related molecules, odontogenic factors, and epithelial mesenchymal transition (EMT)-related molecules.
To the best of our knowledge, this is the first report on the establishment of a CCOC cell line. CCOC-T cells serve as a useful in vitro model for understanding the pathogenesis and nature of MOT.
摘要：
透明细胞牙源性癌（CCOC）是一种罕见的恶性牙源性肿瘤（MOT），其特征是空泡和透明细胞的片状和小叶。为了了解CCOC的生物学，我们建立了一个新的细胞系,CCOC-T,具有来自具有远处转移的下颌骨肿瘤的EWSR1-ATF1融合基因，并表征了该细胞系。
为了检测EWSR1-ATF1融合基因，我们用了三个CCOC病例,包括本案,通过RT-PCR和FISH分析。我们通过检查细胞生长来表征已建立的CCOC-T细胞，牙源性因子和骨相关因子的表达。此外，通过微阵列分析检测CCOC-T细胞的基因表达谱.
组织学，原发肿瘤由索和巢组成，其中包含由纤维间隔分开的透明和鳞片状细胞。此外,观察到成釉细胞岛与栅栏状的外周细胞，表明可能的牙源性起源。该肿瘤表达融合基因EWSR1-ATF1，这是透明透明细胞癌（HCCC）和CCOC潜在病因的基础。我们发现EWSR1-ATF1融合中的断点与HCCC中报道的断点相同。已建立的CCOC-T细胞生长极其缓慢，但是这些细胞表现出高度的侵袭性。此外，CCOC-T细胞表达骨相关分子，牙源性因素，和上皮间质转化（EMT）相关分子。
据我们所知,这是关于建立CCOC细胞系的第一份报告。CCOC-T细胞作为用于理解MOT的发病机理和性质的有用的体外模型。
为了检测EWSR1-ATF1融合基因，我们用了三个CCOC病例,包括本案,通过RT-PCR和FISH分析。我们通过检查细胞生长来表征已建立的CCOC-T细胞，牙源性因子和骨相关因子的表达。此外，通过微阵列分析检测CCOC-T细胞的基因表达谱.
组织学，原发肿瘤由索和巢组成，其中包含由纤维间隔分开的透明和鳞片状细胞。此外,观察到成釉细胞岛与栅栏状的外周细胞，表明可能的牙源性起源。该肿瘤表达融合基因EWSR1-ATF1，这是透明透明细胞癌（HCCC）和CCOC潜在病因的基础。我们发现EWSR1-ATF1融合中的断点与HCCC中报道的断点相同。已建立的CCOC-T细胞生长极其缓慢，但是这些细胞表现出高度的侵袭性。此外，CCOC-T细胞表达骨相关分子，牙源性因素，和上皮间质转化（EMT）相关分子。
据我们所知,这是关于建立CCOC细胞系的第一份报告。CCOC-T细胞作为用于理解MOT的发病机理和性质的有用的体外模型。
