Surgically managed symptomatic ecchordosis physaliphora: a systematic review

PY Toh, Shane Ling, D Wong, J Li Tan

    Research output: Contribution to journalReview articlepeer-review

    Abstract

    Background: Symptomatic ecchordosis physaliphora (EP) is a rare entity and a diagnostic challenge as it is histopathologically indistinguishable from its malignant counterpart, chordoma, and requires a multimodal approach to diagnosis and management. This paper aims to highlight and review surgically managed symptomatic EP, including an evaluation of patient demographics, clinical presentation, classical radiological findings, operative approaches, and outcomes.

    Methods: A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) following a database search in PubMed, Ovid MEDLINE, Ovid EMBASE, and Cochrane Library from inception to January 2023. Studies involving surgically managed cases of EP with diagnosis proven on histopathological and immunohistochemical analysis were included.

    Results: Thirty-two studies were included, with a total of 42 individually reported cases relevant to our inclusion criteria. There was a preponderance towards females, at 64.3% with the mean age being 47.29 years. Clinically, 40.5% of patients presented with a cerebrospinal fluid (CSF) leak and 23.8% had meningitis. The main radiological features of EP included being T1 hypointense, T2 hyperintense, and non-gadolinium enhancing on magnetic resonance imaging (MRI). Twenty-six of the 42 cases underwent an endoscopic endonasal approach to resection and an average follow-up period of 17.34 months post-operatively did not demonstrate any recurrence.

    Conclusions: The management of EP presents a complex challenge due to its histological resemblance to other notochord-derived conditions, notably chordomas. Given the rarity of this entity, our study primarily draws upon case reports and case series, which are recognized to provide a lower level of evidence. However, we believe our study offers essential insights into this condition. Notably, in instances of symptomatic EP, we have found that employing an endoscopic endonasal approach for resection and addressing CSF leaks stands out as a secure and efficacious technique.
    Original languageEnglish
    Article number20
    Number of pages14
    JournalAustralian Journal of Otolaryngology
    Volume6
    DOIs
    Publication statusPublished - 30 Sept 2023

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