颅底及颈部脊索瘤11例临床分析

The study of 11 cases of chordoma in the skull base or neck

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 study the clinical manifestation, histopathological types, radiological characteristics, surgical methods and the prostecdtive efficacy of chordoma in the skull base and neck. Method：Analyzed the characteristics about eleven patients with chordoma in the skull base or neck that we had treated in our department from 1985 to 2003 and introduced a case of a huge chordoma in the neck. All of the patients were treated by surgery. Result：The clinical manifestations of chordoma in the neck or skull base were complex. The first symptoms included the mass in the neck, rhinocleisis, visuognosis fall, tinnitus, hearing loss, and damages of brain nerves. Eight cases were the typical chordoma and the other three were the cartilaginiform chordoma. The dilatant image of parenchyma was the feature in CT scan and the images often had clear confines. The cervical vertebra and the skull base were damaged in the most of the cases. Four patients （4/11） recrudesced in two years after the surgery, and eight of eleven were living more than five years after the surgery. Conclusion： The clinical manifestations of chordoma in the neck or skull base were complex. The cervical vertebra and the skull base.were often damaged in the most of the cases. Surgery is an effective method on chordoma. The surgery path should be selected according to the position of the pathological changes and the size of the mass.