Departments of Otolaryngology and Head and Neck Surgery, Graduate School of Biomedical & Health Sciences, and Applied Life Sciences, Japan.
Departments of Otolaryngology and Head and Neck Surgery, Graduate School of Biomedical & Health Sciences, and Applied Life Sciences, Japan.
Auris Nasus Larynx. 2020 Apr;47(2):299-304. doi: 10.1016/j.anl.2019.03.005. Epub 2019 Mar 21.
Extra-axial chordomas are exceedingly rare malignant tumours. Herein, we present the first case of an extra-axial chordoma in the gingivae.
A 21-year-old man presented with a suspected chordoma in the upper right gingiva. Diagnosis was difficult; however, owing to strong expression of the chordoma marker brachyury, extra-axial chordoma was ultimately diagnosed. The tumour was completely resected without performing a facial incision. To ensure its safety and effectiveness, the surgical procedure was simulated several times before its performance using a three-dimensional (3D) model. Twenty-four months after surgery, the patient remains disease-free.
A diagnosis of extra-axial chordoma can be confirmed by immunohistochemical staining for brachyury.
颅外脊索瘤是极为罕见的恶性肿瘤。在此，我们报告首例发生于牙龈的颅外脊索瘤。
一名 21 岁男性以上颌右牙龈处疑似脊索瘤就诊。诊断困难，但由于脊索瘤标志物 brachyury 强表达，最终诊断为颅外脊索瘤。肿瘤完整切除，未行面部切口。为确保手术的安全性和有效性，术前使用三维（3D）模型多次模拟手术过程。术后 24 个月，患者无疾病复发。
免疫组织化学染色 brachyury 可明确颅外脊索瘤的诊断。
