Lakhani Dhairya A, Martin Daniel
Department of Radiology, West Virginia University, 1 Medical Center Drive, Morgantown, WV 26506.
Radiol Case Rep. 2021 Oct 17;16(12):3937-3939. doi: 10.1016/j.radcr.2021.09.049. eCollection 2021 Dec.
Ecchordosis physaliphora is a rare congenital benign hamartomatous lesion originating from nodal cord remnants. This is histopathologically indistinguishable from chordoma, and hence imaging plays a key role in diagnosis. These lesions are hypointense on T1-weighted and hyperintense on T2-weighted images, and follow CSF signal. In contrast to chordoma, Ecchordosis Physaliphora does not demonstrate contrast enhancement. Here, we present a case of 32-year-old male with no prior medical history, who presented to an outside facility for chronic headache workup and incidentally detected indeterminate lytic defect in the bony clivus with a well demarcated smoothly corticated margin. Further assessment with MRI brain showed findings characteristic of Ecchordosis physaliphora, a benign congenital hamartomatous lesion originating from nodal cord remnants requiring no additional follow-up imaging or intervention.
泡状脊索瘤是一种罕见的先天性良性错构瘤性病变，起源于脊索残余。在组织病理学上，它与脊索瘤无法区分，因此影像学在诊断中起着关键作用。这些病变在T1加权像上呈低信号，在T2加权像上呈高信号，并遵循脑脊液信号。与脊索瘤不同，泡状脊索瘤无强化表现。在此，我们报告一例32岁男性，既往无病史，因慢性头痛在外院进行检查时，偶然发现斜坡骨质有边界清晰、边缘皮质光滑的溶骨性缺损。脑部MRI进一步评估显示为泡状脊索瘤的典型表现，这是一种起源于脊索残余的良性先天性错构瘤性病变，无需进一步的随访影像学检查或干预。
