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目的:探讨发生于耳鼻咽喉科手术部位的颅底及颈部脊索瘤的临床表现,组织学类型,影像学特点,手术方法及远期疗效。方法:回顾性分析11例颅底及颈部脊索瘤患者的临床资料,并介绍1例颈部巨大脊索瘤。11例均行手术治疗。结果:发生于颅底及颈部的脊索瘤临床表现复杂,首发症状以鼻塞、颈部包块、视力下降、耳鸣、耳聋及脑神经受损症状为主。组织学类型:典型脊索瘤8例,软骨性脊索瘤3例。影像学表现:8例行CT检查者表现为软组织肿块影,以膨胀性生长为主,颈椎或颅底多有骨质破坏,肿块与周围软组织边界清楚。2年复发率为36.4%,5年生存率为72.7%。结论:本病临床表现复杂,对颅底及颈椎以外的周围组织以膨胀性压迫为主,对颅底及颈椎骨质以侵蚀性破坏为主;手术治疗效果良好,应根据病变部位及肿瘤大小选择手术径路。复发者仍可再手术,彻底手术可减少复发率。
Objective: To investigate the clinical manifestations, histological types, imaging features, surgical methods and long-term efficacy of skull base and cervical chordoma occurring in the surgical site of otolaryngology. Methods: A retrospective analysis of 11 cases of skull base and cervical chordoma in patients with clinical data, and 1 case of cervical huge chordoma. Eleven patients underwent surgical treatment. Results: The clinical manifestations of chordoma occurred in skull base and neck were complicated. The first symptom was nasal obstruction, neck mass, decreased visual acuity, tinnitus, deafness and impaired cerebral nerves. Histological type: typical chordoma in 8 cases, 3 cases of cartilage chordoma. Imaging findings: 8 cases of CT examination showed soft tissue mass, mainly to the expansion of growth, cervical or skull base more than the destruction of bone, the tumor and the surrounding soft tissue boundaries clear. The 2-year recurrence rate was 36.4% and the 5-year survival rate was 72.7%. Conclusion: The clinical manifestations of this disease is complex, the swelling of the surrounding skull base and other cervical tissue-based, mainly to the skull base and cervical bone erosion-based destruction; surgical treatment is good, should be based on the lesion size and tumor size Choose surgery path. Recurrent patients can still be re-surgery, thorough surgery can reduce the recurrence rate.