Roche P H, Malca S A, Payan M J, Pellet W
Service de Neurochirurgie, Hôpital Sainte-Marguerite, Marseille.
Neurochirurgie. 1995;41(5):353-8.
The authors report a case of chondrosarcoma of the skull base with a myxoïd histology, developed from the right sphenotemporal junction. Twenty six months after enlarged tumour removal, there are no clinical or neuroradiological signs of recurrence. A review of the 180 published cases emphasizes the rarity of this tumour originating of the skull base synchondrosis and occurring in young patients. MRI forms the basis of the topographic work-up but does not formally distinguish it from chordoma. Diagnosis is confirmed by histology which establishes a prognostic scale. Immunohistochemistry eliminates chondroïd chordoma by demonstrating a mesenchymatous phenotype. Only enlarged tumour removal can prolong survival even though improvements have been made in high energy radiation therapy and radiosurgery. Cases of recurrence or rare metastasis are probably due to incomplete exeresis and in most cases linked to high grade histology tumours.
作者报告了一例起源于右蝶颞交界处、组织学为黏液样的颅底软骨肉瘤病例。肿瘤扩大切除术后26个月，无临床或神经放射学复发迹象。对180例已发表病例的回顾强调了这种起源于颅底软骨结合部且发生于年轻患者的肿瘤的罕见性。MRI是影像学检查的基础，但不能将其与脊索瘤明确区分开来。组织学检查确诊并建立了预后分级标准。免疫组织化学通过显示间充质表型排除软骨样脊索瘤。尽管高能放射治疗和放射外科已有改进，但只有扩大肿瘤切除才能延长生存期。复发或罕见转移病例可能是由于切除不彻底，且大多数与高级别组织学肿瘤有关。
