Garofalo Fabio, di Summa Pietro G, Christoforidis Dimitrios, Pracht Marc, Laudato Pietro, Cherix Stéphane, Bouchaab Hanan, Raffoul Wassim, Demartines Nicolas, Matter Maurice
Department of Visceral Surgery, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
Department of Plastic and Reconstructive Surgery, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.
J Surg Oncol. 2015 Oct;112(5):544-54. doi: 10.1002/jso.24026.
Lumbo-sacral chordoma is a rare, slow-growing tumor, arising from embryonic nothocordal remnants. Wide en bloc excision with clear margins remains mandatory to achieve satisfactory recurrence rates and disease-free survival. No chemotherapy has been demonstrated to be effective and radiotherapy is only marginally effective. Tyrosine kinase receptor inhibitors have showed encouraging results in locally advanced and metastatic chordoma. Reconstructive surgery may become very complex. Multidisciplinary approach in tertiary hospitals is always necessary.
腰骶部脊索瘤是一种罕见的、生长缓慢的肿瘤,起源于胚胎脊索残余组织。为了获得满意的复发率和无病生存率,进行切缘阴性的广泛整块切除仍然是必要的。尚无化疗被证明有效,放疗仅略有效果。酪氨酸激酶受体抑制剂在局部晚期和转移性脊索瘤中显示出令人鼓舞的结果。重建手术可能会变得非常复杂。三级医院采用多学科方法总是必要的。