A giant lesion extending from prepontine cistern to the fourth ventricle

Abstract

Because the lesion was adherent to the brainstem, partial resection was performed (Fig. 2), with the help of intraoperative neurophysiological monitoring. Then, pathology revealed chordoma. Chordoma is a rare locally aggressive neoplasm derived from the embryologic notochordal remnants, with incidence of approximately 1/1,000,000 [[1]Persad A.R.L. Mechor B. Starreveld Y. Clival chordoma with brainstem invasion.Can J Neurol Sci. 2018; 45: 235-237Crossref PubMed Scopus (2) Google Scholar]. Chordoma is generally located on the midline including clivus and lumbosacral spine [[2]Walcott B.P. Nahed B.V. Mohyeldin A. Coumans J.V. Kahle K.T. Ferreira M.J. Chordoma: current concepts, management, and future directions.Lancet Oncol. 2012; 13: e69-e76Abstract Full Text Full Text PDF PubMed Scopus (360) Google Scholar]. Symptoms seen with clival chordoma are frequently generated by compression of cranial nerves [[3]Leah P. Dower A. Vescovi C. Mulcahy M. Al Khawaja D. Clinical experience of intracranial chordoma-A systematic review and meta-analysis of the literature.J Clin Neurosci. 2018; 53: 6-12Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar]. Among patients with clival chordoma, intradural extension leading to brainstem compression and extradural extension leading to bony destruction are known phenomena; however, extension from the prepontine cistern to the fourth ventricle is extremely rare. On the preoperative MRI, the clival chordoma mimicks a intra-axial lesion (Fig. 1). Therefore, when MRI shows a neoplasm with severe effacement of the brainstem and similar radiologic features to brainstem tumor, all possibilities including chordoma should be carefully assessed.