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Review
. 2016 Mar 30;8(3):e547.
doi: 10.7759/cureus.547.

Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora

Carlito Lagman  1 , Kunal Varshneya  2 , J Manuel Sarmiento  3 , Alan R Turtz  1 , Rohan V Chitale  4
Affiliations
Review

Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora

Carlito Lagman et al. Cureus. .

Abstract

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.

Keywords: chordoma; ecchordosis physaliphora; notochordal remnant; retroclival lesion.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Brain Radiography for Patient 1
(A) Axial CT (bone window); (B) T1 Axial MRI; (C) T2 Axial MRI; (D) Axial MRI
Figure 2
Figure 2. Brain Radiography for Patient 2
(A) Axial CTA (bone window); (B) Sagittal T1 MRI; (C) Sagittal T2 MRI showing EP and pontine telangiectasia; (D) CTA showing fenestrated basilar artery
See this image and copyright information in PMC

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