脊索瘤好发于颅底和脊柱的浸润性骨肿瘤,起源于残留的胚胎脊索。通常发生在成人,儿童罕见,20岁以下的患者<5%。组织学显示儿童脊索瘤常为低分化,出现细胞异型性、细胞数量增加、核分裂常见,具有侵袭性、转移扩散率高、生存率低等特点。目前分析认为低分化的脊索瘤与SMARCB1/INI1基因缺失有关。为更清楚了解SMARCB1/ Chordoma occurs in the skull base and spine of invasive bone tumors, originated in the residual embryo chordal. Usually occurs in adults, children, rare patients under 20 years of age <5%. Histology showed that children with chordoma often poorly differentiated, there was atypia cells, increased cell numbers, mitotic common, invasive, high rate of metastatic proliferation, low survival rate. The current analysis that poorly differentiated chordoma and SMARCB1 / INI1 gene deletion. To learn more about the SMARCB1 /