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Updated: May 27, 2026

Endoscopic Third Ventriculostomy and Pineal Biopsy from a Single Entry Point

Endoscopic Third Ventriculostomy and Pineal Biopsy from a Single Entry Point

Published on: June 28, 2024

Ectopic pineal chordoma.

Eberval G Figueiredo¹, Wagner M Tavares, Leonardo Welling

¹Division of Neurological Surgery, University of Sao Paulo, School of Medicine, Sao Paulo, Brazil.

Surgical Neurology International

|November 8, 2011

Summary

This case highlights a rare pineal region chordoma metastasizing to the peritoneum via a ventriculoperitoneal shunt. It suggests that intradural extraosseous chordomas may arise independently from notochord remnants within the brain.

Keywords:

Chordoma metastasis notochord pineal region ventriculoperitoneal shunt

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Area of Science:

Neuro-oncology
Pathology

Background:

Chordomas are rare tumors originating from embryonic notochord remnants.
Typically found in sacrococcygeal and clivus regions, they can occur in the neuroaxis.
Intradural extraosseous chordomas may represent a distinct entity.

Observation:

A unique case of a pineal region chordoma with peritoneal metastasis after a ventriculoperitoneal (VP) shunt procedure is presented.
An 18-year-old male with a VP shunt developed abdominal distension and pain.
CT scans revealed masses in the pineal and abdominal regions, confirmed as chordoma by histopathology.

Findings:

The study presents a rare instance of chordoma metastasis through a VP shunt.
Histopathological examination confirmed chordoma in both cranial and abdominal samples.
The patient experienced systemic complications and passed away.

Implications:

This case supports the hypothesis that notochord remnants can persist within the brain.
These remnants may potentially develop into neoplastic lesions, such as chordomas.
Understanding the unique behavior of intradural extraosseous chordomas is crucial for diagnosis and management.