Oral Pathology and Oral Medicine Unit, Department of Oral and Maxillofacial Surgery, Hospital Sultan Abdul Halim, Ministry of Health, 08000, Sungai Petani, Kedah, Malaysia.
Department of Oral and Maxillofacial Surgery, Hospital Sultan Abdul Halim, Ministry of Health, Sungai Petani, Malaysia.
Head Neck Pathol. 2023 Sep;17(3):731-738. doi: 10.1007/s12105-023-01545-x. Epub 2023 Mar 31.
Odontogenic carcinosarcoma (OCS) is an exceptionally rare malignant mixed odontogenic neoplasm, which mostly arises from recurrent benign odontogenic tumour that undergoes malignant transformation.
A literature review was conducted using the keyword of "Odontogenic carcinosarcoma" and all relevant articles were screened. The data collected include demographic profile (age, gender), clinical information (symptoms, location, size), radiologic features, histopathological examination, management, recurrence, metastases, and survival status.
A total of 17 OCS cases including a new case from our hospital. The incidence of OCS was highest in the third decades of life with predilection for male and posterior region of mandible. Clinically, patients may present with swelling and neurological symptoms. Radiographic examination often showed radiolucency with ill-defined border. This tumour demonstrates an aggressive behaviour with reported cases of distant metastases to the lung, lymph nodes, rib, and pelvis. Here, we report an interesting case of OCS in a 38-year-old man with a previous diagnosis of ameloblastoma. The patient was diagnosed with ameloblastoma but refused surgical intervention and returned after 10 years with rapidly enlarging mass on the right side of mandible. Microscopically, the lesion appears as biphasic odontogenic tumour with malignant cytological features seen in both epithelium and mesenchymal components. The spindle to round mesenchymal tumour cells were only positive for vimentin. Ki67 proliferation index was high in both epithelium and mesenchymal components.
This case showed the tendency of untreated ameloblastoma to undergo malignant changes in the long term.
牙源性癌肉瘤(OCS)是一种极为罕见的恶性混合性牙源性肿瘤,主要由复发性良性牙源性肿瘤恶变而来。
使用关键词“牙源性癌肉瘤”进行文献回顾,筛选所有相关文章。收集的数据包括人口统计学特征(年龄、性别)、临床信息(症状、位置、大小)、影像学特征、组织病理学检查、治疗、复发、转移和生存状况。
共纳入 17 例 OCS 病例,包括我院新发病例。OCS 的发病率在 30 多岁最高,男性和下颌后区多见。临床上,患者可能表现为肿胀和神经症状。影像学检查常显示边界不清的透亮区。该肿瘤具有侵袭性行为,有报道称远处转移至肺、淋巴结、肋骨和骨盆。在此,我们报告一例有趣的 OCS 病例,患者为 38 岁男性,既往诊断为造釉细胞瘤。患者最初被诊断为造釉细胞瘤,但拒绝手术干预,10 年后右侧下颌出现快速增大的肿块。显微镜下,病变呈双相性牙源性肿瘤,上皮和间充质成分均有恶性细胞学特征。梭形至圆形间充质肿瘤细胞仅对波形蛋白呈阳性。上皮和间充质成分的 Ki67 增殖指数均较高。
本例显示未经治疗的造釉细胞瘤在长期存在的情况下有恶变的倾向。