PDF(Pubmed)
Abstract：
Sclerosing odontogenic carcinoma (SOC) is an exceedingly rare odontogenic carcinoma known for its locally aggressive yet indolent behavior. There have been no reports of metastasis to distant organs, except a single case involving lymph node metastasis. This report details the case of a 49-year-old female who presented with a well-demarcated radiolucent lesion in the mandible, accompanied by root resorption and tooth displacement. Microscopically, the lesion exhibited a distinctive composition, with two distinct components: cords of epithelium embedded within an abundant collagenous stroma and solid nests of clear polygonal cells surrounded by hyalinized stroma. Notably, the tumor exhibited direct invasion into the submental muscles, accompanied by perineural and vascular invasion, as well as cortical bone loss. Additionally, the clear cells contained diastase-sensitive periodic acid-Schiff-positive granules. Immunohistochemically, the tumor cells displayed positivity for cytokeratin 19 and p63 while testing negative for myoepithelial markers. The Ki-67 index was measured at 23%. Importantly, neitherEWSR1 nor MAML2 rearrangements were detected through fluorescence in situ hybridization (FISH) analysis. Over several years, this patient experienced three instances of local recurrence; notably, four years after the initial surgery, fludeoxyglucose F18-positron emission tomography (18FDG-PET)/CT scans confirmed the presence of pulmonary metastasis. This case presents an unusual histological variation of SOC, marked by vascular invasion, and is notably the first documented case of a fatal outcome in this context.
摘要：
硬化性牙源性癌（SOC）是一种极为罕见的牙源性癌，以其局部侵袭性而懒惰的行为而闻名。没有远处器官转移的报道，除了一例涉及淋巴结转移的病例。本报告详细介绍了一名49岁女性的情况，该女性在下颌骨中出现了清晰的射线可透性病变，伴有牙根吸收和牙齿移位。微观上,病变表现出独特的成分，具有两种不同的成分：嵌入丰富的胶原基质中的上皮组织和由透明基质包围的透明多边形细胞的实心巢。值得注意的是,肿瘤表现出直接侵入下肌，伴有神经周和血管侵犯，以及皮质骨丢失。此外,透明细胞含有淀粉酶敏感的高碘酸希夫阳性颗粒。免疫组织化学，肿瘤细胞显示细胞角蛋白19和p63阳性,而肌上皮标志物检测为阴性.Ki-67指数为23%。重要的是,通过荧光原位杂交（FISH）分析检测到EWSR1或MAML2重排。几年来,该患者经历了三例局部复发；值得注意的是，初次手术四年后，氟脱氧葡萄糖F18正电子发射断层扫描（18FDG-PET）/CT扫描证实存在肺转移。此病例表现出异常的SOC组织学变化，以血管侵入为标志，值得注意的是，在这种情况下，首例有记录的致命结果。
