Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy.
Neurosurg Rev. 2012 Jan;35(1):1-13; discussion 13-4. doi: 10.1007/s10143-011-0354-1. Epub 2011 Oct 18.
Chordomas are extremely rare tumours. They arise in the spheno-occipital region in 35% of cases. Chordomas usually present benign histopathological features but often exhibit a malignant clinical behaviour. Radical surgical removal and high-dose radiation therapy seem to be effective in tumour control and to improve survival rate. Despite the advancements in microsurgical techniques and the development of radiation therapies, clival chordomas still represent a challenge. Nevertheless it appears that chordomas that have been resected to the same extent and that received post-operative radiotherapy might exhibit different rates of regrowth. This result supports the hypothesis that the recurrence rate of chordomas might be dependent on biological variables other than the extent of resection and the post-operative radiotherapy. Genetic and molecular studies on oncogenesis of chordomas are still limited, but they represent the basis for the development of molecular targeted therapies. We present a review of the current knowledge about skull base chordomas biology, therapeutic options and related clinical outcome.
脊索瘤极为罕见。35%的病例起源于蝶枕区。脊索瘤通常具有良性组织病理学特征，但常表现出恶性临床行为。根治性手术切除和高剂量放射治疗似乎能有效控制肿瘤并提高生存率。尽管在显微外科技术和放射治疗方面取得了进展，颅底脊索瘤仍然是一个挑战。尽管如此，接受相同程度的切除和术后放疗的脊索瘤可能会表现出不同的复发率。这一结果支持这样一种假说，即脊索瘤的复发率可能取决于除切除范围和术后放疗之外的生物学变量。脊索瘤发生的遗传和分子研究仍然有限，但它们是开发分子靶向治疗的基础。我们对颅底脊索瘤生物学、治疗选择和相关临床结果的现有知识进行了综述。
