PDF(Pubmed)
Abstract：
BACKGROUND: Sclerosing odontogenic carcinoma is an exceedingly rare gnathic malignancy first described by Koutlas et al. in 2008, and was only recently designated as a distinct pathologic entity by World Health Organization in the 2017 Classification of Head and Neck Tumors. To date, fewer than fifteen cases of this neoplasm have been reported in the English language literature. This tumor is characterized by thin cords, strands, and small nests of epithelium in a densely sclerotic stroma. In some tumor foci, the density of the stroma may be sufficient to compress the epithelial component beyond detection in the absence of immunohistochemistry, thus rendering this entity a particularly challenging diagnosis in small sample sizes.
METHODS: A 55-year-old male presented with an asymptomatic lesion of posterior left maxilla. Cone beam computed tomography (CBCT) demonstrated a large, well-defined bony lesion with scalloped border, spanning from canine to first molar. External root resorption of the adjacent teeth was also noted. Microscopic examination of the biopsy specimen revealed an odontogenic tumor with features consistent with sclerosing odontogenic carcinoma. Immunohistochemical staining was performed to confirm the diagnosis.
RESULTS: The tumor was positive for CK5/6, CK19, E-cadherin, p63 and negative for CK20 and CK7.
CONCLUSIONS: Sclerosing odontogenic carcinoma is a rare, low-grade malignancy of odontogenic origin, which represents a diagnosis of exclusion in many cases. An immunohistochemical profile demonstrating positivity for markers including CK5/6, CK19, p63, and E-cadherin, in addition to a set of pertinent negative findings, can aid in the diagnosis of this tumor. This entity appears to lack metastatic potential despite its locally destructive behavior and a common histologic finding of perineural invasion.
METHODS: A 55-year-old male presented with an asymptomatic lesion of posterior left maxilla. Cone beam computed tomography (CBCT) demonstrated a large, well-defined bony lesion with scalloped border, spanning from canine to first molar. External root resorption of the adjacent teeth was also noted. Microscopic examination of the biopsy specimen revealed an odontogenic tumor with features consistent with sclerosing odontogenic carcinoma. Immunohistochemical staining was performed to confirm the diagnosis.
RESULTS: The tumor was positive for CK5/6, CK19, E-cadherin, p63 and negative for CK20 and CK7.
CONCLUSIONS: Sclerosing odontogenic carcinoma is a rare, low-grade malignancy of odontogenic origin, which represents a diagnosis of exclusion in many cases. An immunohistochemical profile demonstrating positivity for markers including CK5/6, CK19, p63, and E-cadherin, in addition to a set of pertinent negative findings, can aid in the diagnosis of this tumor. This entity appears to lack metastatic potential despite its locally destructive behavior and a common histologic finding of perineural invasion.
摘要：
背景：硬化性牙源性癌是Koutlas等人首次描述的一种极为罕见的颌骨恶性肿瘤。在2008年，直到最近才被世界卫生组织指定为2017年头颈部肿瘤分类的独特病理实体。迄今为止,在英语文献中报道了少于15例这种肿瘤。这个肿瘤的特征是纤细的绳索,股线,和密集硬化基质中的上皮小巢。在一些肿瘤病灶中，在没有免疫组织化学的情况下，基质的密度可能足以压缩上皮成分而无法检测到，因此，在小样本量下，这个实体是一个特别具有挑战性的诊断。
方法：一名55岁男性，表现为无症状的左后上颌骨病变。锥形束计算机断层扫描（CBCT）显示了一个很大的，明确的骨损伤与扇形边界，从犬齿到第一磨牙。还注意到相邻牙齿的外部牙根吸收。活检标本的显微镜检查显示牙源性肿瘤，其特征与硬化性牙源性癌一致。进行免疫组织化学染色以确认诊断。
结果：肿瘤CK5/6、CK19、E-cadherin阳性，p63和CK20和CK7阴性。
结论：硬化性牙源性癌是一种罕见的，牙源性低度恶性肿瘤，这在许多情况下代表了排除的诊断。免疫组织化学谱显示CK5/6、CK19、p63和E-cadherin等标志物阳性，除了一系列相关的负面发现，可以帮助诊断这种肿瘤。尽管该实体具有局部破坏性行为和常见的神经周浸润组织学发现，但该实体似乎缺乏转移潜力。
方法：一名55岁男性，表现为无症状的左后上颌骨病变。锥形束计算机断层扫描（CBCT）显示了一个很大的，明确的骨损伤与扇形边界，从犬齿到第一磨牙。还注意到相邻牙齿的外部牙根吸收。活检标本的显微镜检查显示牙源性肿瘤，其特征与硬化性牙源性癌一致。进行免疫组织化学染色以确认诊断。
结果：肿瘤CK5/6、CK19、E-cadherin阳性，p63和CK20和CK7阴性。
结论：硬化性牙源性癌是一种罕见的，牙源性低度恶性肿瘤，这在许多情况下代表了排除的诊断。免疫组织化学谱显示CK5/6、CK19、p63和E-cadherin等标志物阳性，除了一系列相关的负面发现，可以帮助诊断这种肿瘤。尽管该实体具有局部破坏性行为和常见的神经周浸润组织学发现，但该实体似乎缺乏转移潜力。
