Evaluation of Long-Term Follow-Up in Ecchordosis Physaliphora versus Chordoma

Ecchordosis physaliphora (EP) is a non-neoplastic notochord remnant with limited literature. We present a review on surgically resected clival EP to evaluate if available follow-up is adequate to distinguish EP from chordomas.

A systematic literature review was completed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Case reports or series of adults with histopathologic and radiographic findings of surgically resected EP were included. Articles including pediatric patients, systematic reviews, chordomas, and without microscopic or radiographic confirmation, or the surgical approach, were excluded. Corresponding authors were contacted twice to further evaluate outcomes.

Eighteen articles were included (n = 25 patients; mean age 47.5 years ± 12.6 [standard deviation] months). All patients had symptomatic, surgically resected EP, with cerebrospinal fluid leak or rhinorrhea the most common symptom (48%). All but 3 had gross total resection, with endoscopic endonasal transsphenoidal transclival the most common approach (80%). All but 3 reported immunohistochemistry findings, with physaliphorous cells the most common. All but 5 patients had definitive follow-up (80%), with average of 19.5 ± 17.2 months. One corresponding author reported longer-term follow-up for 1 patient (57 months). No recurrence or malignant transformation was reported. Mean time to clival chordoma recurrence (53.9 ± 26.8 months) was also evaluated in a review of 8 studies.

Mean follow-up for resected EP was almost 3 times shorter than mean time to recurrence of chordomas. Available literature is likely inadequate to confirm the suspected benign nature of EP especially in reference to chordoma, precluding treatment and follow-up recommendations.