Yeh Chi-Yuan
Department of Radiation Oncology, Tungs' Taichung Metroharbor Hospital, Taichung 43503, Taiwan, R.O.C.
Mol Clin Oncol. 2021 Apr;14(4):84. doi: 10.3892/mco.2021.2246. Epub 2021 Feb 26.
Chordomas are slow-growing aggressive tumors that account for 1-4% of all bone tumors. The anatomical distribution of chordomas includes 50-60% in the sacrococcygeal region, 25-30% in the skull base and 15% in the mobile spine. Virchow was the first to describe and term these tumors as 'ecchordosis physaliphora' in 1857, and Muller established their notochordal origin in 1895. Extraosseous chordomas of the nasopharynx are very rare, and they exhibit similarities with other lesions of the nasopharynx, presenting as a soft tissue mass. Gross total resection combined with postoperative radiotherapy offers the best chance of long-term control. We herein present the case of a 63-year-old female patient with complaints of left temporal headaches, dizziness, left nasal obstruction, left maxillary area numbness, left ear hearing loss and swallowing difficulty. Computed tomography imaging examination revealed an 8.2x3.2x5.7-cm space-occupying lesion with central necrosis in the nasopharynx and oropharynx, partially occluding the pharyngeal lumen; the mass had infiltrated the left parapharyngeal space, the left medial and lateral pterygoid muscle and the left parotid gland, with bone erosion of the left mandible. The patient was diagnosed with extraosseous chordoma of the nasopharynx, conventional type, stage IIB. The patient underwent surgery and high-dose radiotherapy and local control of the chordoma was achieved. However, the patient succumbed to a lung metastasis. The details of the case are discussed, and a review of the current medical literature is presented to provide an updated discussion on the current status of nasopharyngeal chordoma research.
脊索瘤是生长缓慢的侵袭性肿瘤，占所有骨肿瘤的1%-4%。脊索瘤的解剖分布包括：50%-60%位于骶尾区域，25%-30%位于颅底，15%位于活动脊柱。1857年，魏尔啸首次描述并将这些肿瘤命名为“泡状脊索瘤”，1895年，米勒确定了它们的脊索起源。鼻咽部的骨外脊索瘤非常罕见，与鼻咽部的其他病变相似，表现为软组织肿块。根治性手术联合术后放疗提供了最佳的长期控制机会。我们在此报告一例63岁女性患者，主诉左侧颞部头痛、头晕、左侧鼻塞、左侧上颌区域麻木、左耳听力减退和吞咽困难。计算机断层扫描成像检查显示，鼻咽部和口咽部有一个8.2×3.2×5.7厘米的占位性病变，中央坏死，部分阻塞咽腔；肿块已侵犯左侧咽旁间隙、左侧翼内肌和翼外肌以及左侧腮腺，左侧下颌骨有骨质侵蚀。该患者被诊断为常规型IIB期鼻咽部骨外脊索瘤。患者接受了手术和高剂量放疗，脊索瘤得到了局部控制。然而，患者死于肺转移。本文讨论了该病例的详细情况，并对当前医学文献进行了综述，以提供关于鼻咽部脊索瘤研究现状的最新讨论。
