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Case Reports
. 2021 Oct 17;16(12):3937-3939.
doi: 10.1016/j.radcr.2021.09.049. eCollection 2021 Dec.

Ecchordosis physaliphora: Case report and brief review of the literature

Dhairya A Lakhani  1 , Daniel Martin  1
Affiliations
Case Reports

Ecchordosis physaliphora: Case report and brief review of the literature

Dhairya A Lakhani et al. Radiol Case Rep. .

Erratum in

Abstract

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.

Keywords: Chordoma; Ecchordosis Physaliphora; Notochord.

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Figures

Fig 1
Fig. 1
CT Brain without contrast showed an indeterminate lytic defect in the bony clivus with a well demarcated smoothly corticated margin (arrow). This was reported as concerning for malignancy, with recommendation for further evaluation with MRI brain and referral to our tertiary care center for further workup.
Fig 2
Fig. 2
MRI brain with and without contrast was performed at our facility. This demonstrated a well-defined midline retroclival 5 mm x 12 mm cystic lesion (arrow) with hypointense signal on T1-weighted (A, B) and hyperintense signal on T2-weighted images (C), and followed CSF signal on T2-Fluid-attenuated inversion recovery (T2-FLAIR) images (D). Further, this lesion did not restrict diffusion (E) and did not demonstrate abnormal enhancement (F). Based on the midline location and characteristic imaging appearance, the findings were reported as compatible with Ecchordosis physaliphora, a benign congenital hamartomatous lesion originating from nodal cord remnants requiring no additional follow-up imaging or intervention
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References

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