Case ReportA Hemorrhagic Clival Chordoma with a Long Progression-Free Survival
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.
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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.