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Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity

  • Original Article - Pituitaries
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Abstract

Objective

This paper highlights the management of 5 patients affected by symptomatic ecchordosis physaliphora (EP), treated via endoscopic endonasal transsphenoidal-transclival approach and contextual multilayer skull base reconstruction. A detailed analysis of each case is provided, along with the review of the current body of literature.

Methods

A retrospective review of patients treated by means of endoscopic endonasal approach for EP from 2010 to 2020 in the Otolaryngology and Neurosurgery Departments of a tertiary-care referral center for endoscopic skull base surgery was analyzed. Only adult patients with a definitive histopathological and immunohistochemical diagnosis of EP were included in the study. A systematic literature review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed for EP.

Results

Five cases of EP were retrieved and included in the study. Four patients presented with CSF leakage: in two cases after minor head trauma, in one case with associated bacterial meningitis, and in one case as only referred symptom. One patient complained diplopia due to VI cranial nerve palsy. No complications or recurrences of the disease were observed after a median follow-up of 37.2 months (range, 18–72 months). A total of 27 studies were identified with the systematic literature review, encompassing 30 patients affected by symptomatic EP who were addressed to surgical treatment. Twenty-five patients underwent complete surgical removal of the EP, while in 5 cases, only subtotal resection was performed.

Conclusions

EP might result in a “locus minoris resistentiae” of the skull base, predisposing, in rare cases, to CSF leakage and meningitis, spontaneously or after minor trauma. In cases of symptomatic EP, endoscopic endonasal transsphenoidal-transclival approach represents a safe and effective technique for both EP resection and contextual skull base reconstruction.

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Abbreviations

EP:

Ecchordosis physaliphora

CT:

Computed tomography

MRI:

Magnetic resonance image

Gd:

Gadolinium

BNCT:

Benign notochord cell tumor

CH:

Chordoma

PRISMA:

Preferred Reporting Items for Systematic Reviews and Meta-Analyses

CSF:

Cerebrospinal fluid

EMA:

Epithelial membrane antigen

CK:

Cytokeratin

CEA:

Carcinoembryonic antigen

EEA:

Endoscopic endonasal approach

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Author information

Authors and Affiliations

  1. Scuola di Specializzazione in Neurochirurgia, Università di Pavia, Pavia, Italy

    Pierlorenzo Veiceschi

  2. Division of Neurosurgery, Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy

    Pierlorenzo Veiceschi, Edoardo Agosti & Davide Locatelli

  3. Division of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy

    Alberto Daniele Arosio, Andrea Pistochini & Paolo Castelnuovo

  4. Department of Biotechnology and Life Sciences, Head and Neck Surgery and Forensic Dissection Research Center (HNS&FDRc), University of Insubria, Varese, Italy

    Alberto Daniele Arosio, Maurizio Bignami, Andrea Pistochini, Paolo Castelnuovo & Davide Locatelli

  5. Division of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, Como, Italy

    Maurizio Bignami

  6. Department of Surgical Pathology, ASST Sette Laghi, Ospedale di Circolo e Fondazione Macchi, Varese, Italy

    Michele Cerati

Authors
  1. Pierlorenzo Veiceschi
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  2. Alberto Daniele Arosio
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Correspondence to Edoardo Agosti.

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All patients involved in this study signed a consent form to publish their data or clinical images whenever useful.

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Veiceschi, P., Arosio, A.D., Agosti, E. et al. Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity. Acta Neurochir 163, 2475–2486 (2021). https://doi.org/10.1007/s00701-021-04857-5

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  • DOI: https://doi.org/10.1007/s00701-021-04857-5

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