Intradural Clival Chordoma and Ecchordosis Physaliphora

Abstract

OBJECTIVE Purely intradural clival chordomas are rare neoplasms, and only a few cases have been reported. The reported cases present features similar to ecchordosis physaliphora, which is a notochordal remnant. We describe these 2 entities and their differential diagnoses, clinical courses, and management. This is the first reported case to be treated using a neuroendoscopic technique. CLINICAL PRESENTATION A 60-year-old man presenting with memory loss underwent magnetic resonance imaging, which revealed an intradural retroclival mass without bone involvement. INTERVENTION The patient underwent an endoscopic transsphenoidal-transclival procedure with subtotal removal of the tumor. Histological findings confirmed the diagnosis of a chordoma. CONCLUSION Even if some parameters exist for a differential diagnosis, ecchordosis physaliphora and intradural chordoma may represent different aspects of the spectrum of the same pathology. Intradural clival chordomas have a better prognosis with respect to classic chordomas. Therefore, in subtotal removal such as that performed in our case, postoperative radiation therapy should be performed only if a regrowth of the remnant is seen during neuroradiological follow-up.

Intradural prepontine chordoma in an 11-year-old boy. A case report

“…EPs are typically found in the prepontine cistern, attached to the dorsal clivus with a small pedicle [ 13 , 25 ]. Other distinctive features are that EPs generally occur in younger people compared to intradural chordomas and that EPs show no enhancement after gadolinium administration on MR imaging, whereas intradural chordomas show a variable enhancement [ 7 , 17 ]. Although differentiating between symptomatic EPs and intradural chordomas based on histology is difficult and debated, Yamaguchi et al [ 26 ] suggested some histological differentiating features.…”

“…Although differentiating between symptomatic EPs and intradural chordomas based on histology is difficult and debated, Yamaguchi et al [ 26 ] suggested some histological differentiating features. Symptomatic EPs and intradural chordomas tend to show a similar benign behaviour with a very low risk of tumour recurrence after surgical resection [ 7 ]. Chang et al [ 5 ] suggest that EPs and intradural chordomas exist in a continuum from benign hamartomas to aggressive typical chordomas.…”

A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery

“…reported similar approach by ETSS in the case of intradural chordoma. [5] By ETSS, the surgeon can approach a retroclival prepontine lesion without retracting the brain. To expose the mass, the clivus must be opened, which is a minimally invasive approach.…”

“…Interventions of intradural chordomas should be aimed at total removal of the tumor, which may require adjuvant radiotherapy for the residual tissue. [45] Using MRI, Mehnert et al . reported differential diagnoses between EP and a chordoma and found that all EP cases showed no contrast enhancement by gadolinium-enhanced T1-weighted MRI.…”

“…[3–513] However, symptomatic EP cases are extremely rare, as only 11 cases have been reported to date, most of which were managed by resection via craniotomy. [1246–810–121415] Here we report, a rare case of symptomatic EP managed successfully by endoscopic endonasal trans-sphenoidal surgery (ETSS) without resection via craniotomy.…”