Elsevier

World Neurosurgery

Volume 105, September 2017, Pages 1042.e1-1042.e4
World Neurosurgery

Case Report
A Hemorrhagic Clival Chordoma with a Long Progression-Free Survival

https://doi.org/10.1016/j.wneu.2017.06.169Get rights and content

Background

Chordomas and ecchordosis physaliphora may on rare occasions present with intracranial hemorrhage. Their distinction usually relies on the results of the Ki-67 proliferative index, with a result lower than 1% favoring ecchordosis physaliphora. Intracranial hemorrhagic chordomas have been linked to unfavorable prognosis, due to acute neurologic deterioration and death, or progression after treatment. To the best of our knowledge, this is the first report of a patient with an intracranial hemorrhagic chordoma who had a long progression-free survival.

Case Description

A 67-year-old woman presented with a large hemorrhagic clival tumor that was resected through an endonasal endoscopic approach. Physallipharous cells interspersed in a myxoid matrix, positivity for S-100, cytokeratin, and epithelial membrane antigen were found, along with an extremely low Ki-67 index. Imaging findings of bone erosion, a large size, and enhancement favored the diagnosis of chordoma. The patient received adjuvant stereotactic radiotherapy and has remained disease free after 4 years.

Conclusions

Although hemorrhagic intracranial chordomas have been linked to unfavorable outcomes, our case demonstrates that they may have a low proliferative index, and a long progression-free survival may be seen.

Introduction

Chordomas and ecchordosis physaliphora (EP) are lesions that arise from remnants of the notochord,1 and both may rarely present with intracranial hemorrhage.2, 3 Clear-cut differentiating features between these 2 entities are lacking, and the diagnosis of ecchordosis is supported by a Ki-67 index lower than 1% and lack of bone erosion and enhancement with gadolinium, among other imaging features.4 Intracranial hemorrhagic chordomas reported to date had a Ki-67 index greater than 1%, due to their highly proliferative nature, and an unfavorable course either due to death or due to tumor progression after treatment.3, 5, 6, 7, 8, 9, 10 Herein, we present the case of a patient with a large clival hemorrhagic chordoma, with an extremely low Ki-67 index, who had a long progression-free survival, which we believe is the first report of such occurrence.

Section snippets

Case Report

A 67-year-old woman presented with headache and dizziness after a minor fall. Her neurologic examination was completely normal, and there was no evidence of a cerebrospinal fluid leak. Computed tomography scan of the head demonstrated an erosive lesion arising from the clivus, with an acute intratumoral hemorrhage (Figure 1). Magnetic resonance imaging of the head demonstrated a clival lesion, hyperintense on T1- and T2-weighted images, with extension into the sphenoid sinus and the prepontine

Discussion

Chordomas and EP may rarely lead to intracranial hemorrhage,2, 3, 10, 11 and their distinction may become a challenge because of their pathologic similarities, because both arise from remnants of the notochord. EP is usually a small (<6 cm3) gelatinous lesion, arising from a small, well-delineated and midline clival bone defect.4, 12 No enhancement and no major bony erosion are seen.13 Occasionally, EP may rarely grow and become symptomatic.14 Chordomas are seen as centrally located, expansive

Conclusions

The mere presence of hemorrhage does not necessarily indicate aggressiveness in hemorrhagic chordomas, as they may have a very low Ki-67 proliferative index, and a long-term progression free-survival may be seen.

References (27)

  • A.D. Levi et al.

    Clival chordoma presenting with acute brain stem hemorrhage

    Can J Neurol Sci

    (1991)
  • R. Nakau et al.

    Subarachnoid hemorrhage associated with clival chordoma—case report

    Neurol Med Chir (Tokyo)

    (2003)
  • T. Uda et al.

    An intradural skull base chordoma presenting with acute intratumoral hemorrhage

    Neurol India

    (2006)
  • Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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