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Myxopapillary ependymoma of the fourth ventricle: report of a unique case and review of the literature
Author(s) -
Li Jianyi,
Aizenberg Michele,
Powell Suzanne Z,
McCutcheon Ian E,
Fuller Gregory N
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a395
Subject(s) - filum terminale , ependymoma , fourth ventricle , anatomy , ependymal cell , pathology , medicine , hyaline , spinal cord , third ventricle , immunohistochemistry , psychiatry
Background: Myxopapillary ependymoma (MPE) is a distinctive low‐grade (WHO grade I) ependymal neoplasm that arises almost exclusively from the spinal cord filum terminale. Exceptionally, MPE has been reported at CNS sites outside of the filum terminale. There have been only two cases of MPE arising in the posterior fossa (Tseng et al. 2004; Lim et al. 2006). We report here a confirmatory third case of MPE arising in the fourth ventricle. Case report: A 72‐year‐old man presented with a 6‐month history of gait disorder and ataxia. MRI studies revealed a 3.5 x 3.2 x 3.9 cm mass in the fourth ventricle. H&E‐stained cytologic smear preparations demonstrated glial cells with relatively uniform, bland nuclei and long cytoplasmic processes, and scattered patches of bluish‐purple, acellular, myxoid material in the background. Occasional prominent intracytoplasmic brightly eosinophilic hyaline globules surrounded by a clear halo were present. Tissue sections showed features of myxopapillary ependymoma, including prominent alcian blue‐positive microcysts and myxoid cuffs surrounding blood vessels. Mitoses were rare. Tumor cells were strongly positive for GFAP, and prominent EMA positivity was present in perinuclear dot‐like and ring patterns. Ultrastructural examination demonstrated characteristic features of ependymal differentiation. Conclusion: We report here the third case of MPE of the fourth ventricle. The very limited amount of data in the literature suggest that extended recurrence‐free survival is possible after surgical resection. Additional experience is required to further elucidate the clinicopathologic features of MPE arising in the fourth ventricle.