An unusual case of oropharyngeal chordoma: A case report and literature review.
An unusual case of oropharyngeal chordoma: A case report and literature review.
复制标题
DOI：
10.1097/md.0000000000008963
复制
发表时间：
2017-12
期刊：
影响因子：
1.6
通讯作者：
Yang H
中科院分区：
文献类型：
--
作者：
Li X;Wang Y;Wang F;Li B;Sun S;Yang H
脊索瘤是一种罕见的恶性肿瘤，起源于脊索组织沿着颅尾轴的不完全退化。发生在非典型位置的脊索瘤称为异位脊索瘤，如口咽脊索瘤（OC）。OCs非常罕见;迄今为止只有11例报告。术前诊断具有挑战性，因此准确的诊断是基于术后病理检查结果和免疫组化。虽然部分患者进行了手术治疗和放射治疗，但5年生存率很低。越来越多的脊索瘤研究建立在分子生物学的基础上，以提高生存率，靶向治疗可能是未来的一种新的治疗方法。患者出现左口咽肿块，1年前开始缓慢增大。在此期间，他报告了异物感和发音困难。患者最初被诊断为神经源性肿瘤。常规术后病理显示肿块与脉络膜一致。行肿块切除术。初次手术后一年，磁共振成像显示左侧咽后间隙和斜坡处有块状信号图像。患者出现OC复发。手术切除是治疗OC的主要手段，术后辅助放疗也很重要。了解本报告中描述的不寻常病例可能有助于诊断OC，靶向治疗的发展可能有助于临床医生为OC患者提供新的治疗方法。
Chordomas are rare malignant neoplasms derived from incomplete regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that develop in an atypical position are called ectopic chordomas, such as oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases have been reported to date. Preoperative diagnosis is challenging, and an accurate diagnosis thus is based on postoperative pathologic examination findings and immunohistochemistry. Although surgical therapy and radiotherapy is performed in some patients, the 5-year survival rate is low. Increasingly more studies of chordomas have been based on molecular biology to increase the survival rate, and targeted therapy could be a new therapy in the future. The patient presented with a left oropharyngeal mass that had begun slowly enlarging 1 year previously. He reported a foreign body sensation and dysphonia during this time period. The patient was initially diagnosed with a neurogenic tumor. Routine postoperative pathology showed that the mass was consistent with a chordoma. Mass resection was performed. One year after the initial surgery, magnetic resonance imaging revealed block signal images at the left retropharyngeal space and clivus. The patient developed recurrence of the OC. Surgical resection is the mainstay of treatment for OC, and postoperative adjuvant radiotherapy is also important. An understanding of the unusual case described in this report may be helpful in diagnosing OC, and development of targeted therapy may help clinicians to provide novel treatment for patients with OC.