A Hemorrhagic Clival Chordoma with a Long Progression-Free Survival.

Abstract

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. 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. 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.