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.
Highlights
Ecchordosis physaliphora (EP) is a benign, congenital lesion representing a notochordal remnant thought to arise by perforation of ectopic nests through the axial skeleton
The diagnostic criteria we propose is based on features consistently cited in the literature; it is glaringly obvious that in order to meet histological and immunohistochemical criteria, one must obtain a tissue sample via biopsy
We reviewed many case reports that described novel techniques of resection, analysis of tissue was often performed after near-total or gross resection of the masses
Summary
Ecchordosis physaliphora (EP) is a benign, congenital lesion representing a notochordal remnant thought to arise by perforation of ectopic nests through the axial skeleton It occurs at the level of the clivus and sacrum where a convoluted course provides a detour from these cells’ natural embryologic path [1]. We believe these two tools can help guide neurosurgeons in the management and prevention of potentially catastrophic events (e.g., sudden death due to subarachnoid hemorrhage) that could occur when one favors a conservative approach with a high-grade EP or mistakenly diagnoses EP in the context of a malignant chordoma [3]
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