Department of Oral and Maxillofacial Surgery, Hannover Medical School, Hannover, Germany.
Department of Pathology, Hannover Medical School, Hannover, Germany.
Int J Oral Maxillofac Surg. 2022 Dec;51(12):1525-1529. doi: 10.1016/j.ijom.2022.02.017. Epub 2022 Mar 23.
Extra-axial chordomas are rare malignant tumours. As a subcategory of axial chordomas, these tumours arise outside the axial skeleton. This report describes the case of a 13-year-old male patient with a gingival mass in the left upper jaw, who was referred to Hannover Medical School with a preliminary diagnosis of a calcifying epithelial odontogenic tumour (CEOT). Pathological examination of the enucleated tumour led to the final diagnosis of a chordoma. Thereafter, a stepwise radical resection was performed with the aim of complete resection of the tumour with wide safety margins. The main tumour mass was found to be located on the maxillary gingiva, with focal infiltration into the maxillary bone. Following resection, reconstruction was performed with a free latissimus dorsi flap. Follow-up after 1 year revealed no signs of recurrence or metastasis. This case highlights that although extremely rare, extra-axial chordoma may occur at sites distant from the midline and as such must be included in the differential diagnosis of bone and soft tissue tumours in the maxillofacial region.
颅外脊索瘤是一种罕见的恶性肿瘤。作为轴性脊索瘤的一个亚类，这些肿瘤发生在轴性骨骼之外。本报告描述了一例 13 岁男性患者，其左上颚有牙龈肿块，最初被诊断为钙化上皮性牙源性肿瘤（CEOT），后转诊至汉诺威医学院。被切除肿瘤的病理检查最终诊断为脊索瘤。此后，采用逐步根治性切除术，旨在通过广泛安全的切缘实现肿瘤的完全切除。主要肿瘤块位于上颌牙龈，局部浸润上颌骨。切除后，采用游离背阔肌皮瓣进行重建。1 年后的随访未发现复发或转移的迹象。本例提示颅外脊索瘤虽然极其罕见，但可能发生在远离中线的部位，因此必须纳入颌面骨和软组织肿瘤的鉴别诊断。
