Ecchordosis physaliphora (EP) is a benign notochordal remnant derived from ectopic nests found along the craniospinal axis. It typically presents asymptomatically and is diagnosed using classic radiologic features, particularly location, T1-hypointensity, T2-hyperintensity, and lack of enhancement following gadolinium (Gd) contrast administration. Distinguishing EP from its malignant counterpart, chordoma, is of paramount importance, given the aggressive nature of the latter. Advances in imaging and immunohistochemistry have aided in diagnosis to an extent but, to our knowledge, identification of the genetic fingerprint of EP has yet to take place. Further cytological analysis of these lesions in search of a genetic link is warranted. We propose here a set of diagnostic criteria based on features consistently cited in the literature. In this literature review, 23 case reports were identified and collated into a summary of symptomatic cases of ecchordosis physaliphora. An illustrative case report of two patients was also included.
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http://dx.doi.org/10.7759/cureus.547 | DOI Listing |
Neuroradiol J
November 2024
Department of Radiology, AZ Maria Middelares, Ghent, Belgium.
Ecchordosis physaliphora (EP) is a benign notochordal remnant most commonly encountered in the skull base. In opposition to typical cases of its invasive counterpart, that is, chordoma, EP does not show T1-enhancement. Now, we describe three patients with EP, discovered on delayed contrast-enhanced 3D FLAIR performed for endolymphatic hydrops imaging in suspected Menière's disease.
View Article and Find Full Text PDFWorld Neurosurg
November 2024
Department of Neurosurgery, Erciyes University School of Medicine, Kayseri, Turkey.
Neurosurg Focus
May 2024
1Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, Salford, Manchester.
Objective: The aim of this study was to describe the natural history of incidental benign-appearing notochordal lesions of the skull base with specific attention to features that can make differentiation from low-grade chordoma more difficult, namely contrast uptake and bone erosion.
Methods: In this retrospective case series, the authors describe the clinical outcomes of 58 patients with incidental benign-appearing notochordal lesions of the clivus, including those with minor radiological features of bone erosion or contrast uptake.
Results: All lesions remained stable during a median follow-up of almost 3 years.
Cureus
November 2023
Department of Internal Medicine, Taif University, Taif, SAU.
Ecchordosis physaliphora (EP) is a rare benign lesion arising from embryonic notochordal remnants, typically located in the retroclival region. This case report presents a 46-year-old male patient experiencing intermittent headaches and occipital pain. Imaging revealed a well-defined, smoothly corticated bony lesion on the left side of the clivus, accompanied by a characteristic bony stalk devoid of any aggressive features.
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