Brown R V, Sage M R, Brophy B P
Department of Radiology, Flinders Medical Centre, Bedford Park, South Australia.
AJNR Am J Neuroradiol. 1990 Jan-Feb;11(1):121-4.
Chordomas are rare neoplasms arising from remnants of the embryologic notochord. Although typically midline in site, intracranial tumors may arise off the midline, and a significant proportion of these arise unilaterally in the petrous bone. Three examples of this entity are presented, all demonstrating typical radiologic features. Dendritic branches of the cranial end of the notochord ramifying in the skull base are thought to provide the embryologic basis for this tumor. The CT and MR features demonstrated are similar to those of the more common midline chordomas. CT demonstrates a well-defined extraaxial soft-tissue mass associated with foci of calcification and bone destruction and occurring at a site corresponding with the embryologic distribution of notochordal material. On MR, prolonged T1 and T2 relaxation times result in hypointensity on T1-weighted images and marked hyperintensity on T2-weighted images. Signal voids result from the foci of calcification while hemorrhage may also lead to inhomogeneity.
脊索瘤是一种起源于胚胎脊索残余组织的罕见肿瘤。虽然肿瘤通常位于中线部位,但颅内肿瘤也可能偏离中线出现,其中相当一部分单侧发生于岩骨。本文展示了该实体的三个病例,均表现出典型的影像学特征。脊索颅端的树突状分支在颅底呈放射状分布,被认为是该肿瘤的胚胎学基础。所展示的CT和MR特征与更常见的中线脊索瘤相似。CT显示一个边界清晰的轴外软组织肿块,伴有钙化灶和骨质破坏,位于与脊索物质胚胎分布相对应的部位。在MR上,T1和T2弛豫时间延长导致T1加权像上呈低信号,T2加权像上呈明显高信号。钙化灶表现为信号缺失,而出血也可能导致信号不均匀。