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Abstract：
BACKGROUND: Clear cell odontogenic carcinoma (CCOC) is an odontogenic carcinoma characterized by sheets and islands of vacuolated and clear cells. The diagnosis of atypical CCOC can pose a challenge when tumor cells deviate from their characteristic clear morphology, even with the aid of genetic profiling for CCOC identification.
METHODS: In this manuscript, we detailed the inaugural instance of a recurrently recurring clear cell odontogenic carcinoma (CCOC) with pronounced squamous differentiation in a 64-year-old male. The primary tumor in this individual initially displayed a biphasic clear cell phenotype. However, subsequent to the third recurrence, the clear tumor cells were entirely supplanted by epidermoid cells characterized by eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. Notable aggressive attributes such as necrosis, conspicuous cytological malignancy, perineural dissemination, and vascular invasion were noted. Additionally, the tumor progressed to manifest lung metastases. The tumor cells exhibited positive immunoreactivity for AE1/AE3, KRT19, Pan-CK, EMA, P40, P63, CK34βE12, and P53, while they tested negative for CK35βH11, KRT7, S-100, and neuroendocrine markers. The Ki-67 proliferation index was calculated at an average of 15%. Furthermore, FISH analysis unveiled the presence of the EWSR1::ATF1 gene fusion.
CONCLUSIONS: This case illustrated a rare and aggressive case of CCOC characterized by significant squamous differentiation upon recurrence of the tumor.
METHODS: In this manuscript, we detailed the inaugural instance of a recurrently recurring clear cell odontogenic carcinoma (CCOC) with pronounced squamous differentiation in a 64-year-old male. The primary tumor in this individual initially displayed a biphasic clear cell phenotype. However, subsequent to the third recurrence, the clear tumor cells were entirely supplanted by epidermoid cells characterized by eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. Notable aggressive attributes such as necrosis, conspicuous cytological malignancy, perineural dissemination, and vascular invasion were noted. Additionally, the tumor progressed to manifest lung metastases. The tumor cells exhibited positive immunoreactivity for AE1/AE3, KRT19, Pan-CK, EMA, P40, P63, CK34βE12, and P53, while they tested negative for CK35βH11, KRT7, S-100, and neuroendocrine markers. The Ki-67 proliferation index was calculated at an average of 15%. Furthermore, FISH analysis unveiled the presence of the EWSR1::ATF1 gene fusion.
CONCLUSIONS: This case illustrated a rare and aggressive case of CCOC characterized by significant squamous differentiation upon recurrence of the tumor.
摘要：
背景：透明细胞牙源性癌（CCOC）是一种牙源性癌，其特征是空泡和透明细胞的片状和岛状。当肿瘤细胞偏离其特征性的清晰形态时，非典型CCOC的诊断可能会带来挑战。即使借助遗传谱分析进行CCOC鉴定。
方法：在本手稿中，我们详细介绍了在一名64岁男性中复发的透明细胞牙源性癌（CCOC）的首例病例，该病例具有明显的鳞状分化。该个体中的原发性肿瘤最初表现出双相透明细胞表型。然而,在第三次复发之后，透明的肿瘤细胞被以嗜酸性细胞质为特征的表皮样细胞完全取代，囊泡染色质，和突出的核仁。显著的侵略性属性，如坏死，明显的细胞学恶性肿瘤，神经周传播，并注意到血管侵犯。此外,肿瘤进展为明显的肺转移。肿瘤细胞对AE1/AE3、KRT19、Pan-CK、EMA,P40，P63，CK34βE12和P53，而它们对CK35βH11，KRT7，S-100和神经内分泌标志物的检测均为阴性。计算的Ki-67增殖指数平均为15%。此外，FISH分析揭示了EWSR1::ATF1基因融合的存在。
结论：该病例说明了一例罕见且侵袭性的CCOC病例，其特征是肿瘤复发时显著鳞状分化。
方法：在本手稿中，我们详细介绍了在一名64岁男性中复发的透明细胞牙源性癌（CCOC）的首例病例，该病例具有明显的鳞状分化。该个体中的原发性肿瘤最初表现出双相透明细胞表型。然而,在第三次复发之后，透明的肿瘤细胞被以嗜酸性细胞质为特征的表皮样细胞完全取代，囊泡染色质，和突出的核仁。显著的侵略性属性，如坏死，明显的细胞学恶性肿瘤，神经周传播，并注意到血管侵犯。此外,肿瘤进展为明显的肺转移。肿瘤细胞对AE1/AE3、KRT19、Pan-CK、EMA,P40，P63，CK34βE12和P53，而它们对CK35βH11，KRT7，S-100和神经内分泌标志物的检测均为阴性。计算的Ki-67增殖指数平均为15%。此外，FISH分析揭示了EWSR1::ATF1基因融合的存在。
结论：该病例说明了一例罕见且侵袭性的CCOC病例，其特征是肿瘤复发时显著鳞状分化。
