Combined Occurrence of Mucin-Producing Ependymoma and Neuroepithelial (Colloid) Cysts

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Abstract
A three-month-old girl had a generalized convulsion which later recurred intermittently. She eventually suffered a right hemiparesis and died at two years of age. An ependymoma, probably congenital, with papillary formation and an excessive production of mucin was present, both above and below the tentorium. Part of the supratentorial tumor filled the left lateral and third ventricles. The choroid plexus of the left lateral ventricle contained multiple small neuroepithelial (colloid) cysts. Although both these lesions of neuroepithelial origin have been encountered throughout the neuraxis, their combined occurrence is rare. Multiple neuroepithelial cysts should be differentiated from cystic ependymoma and follicular adenoma of the choroid plexus. Neuroepithelial cells, both normal and abnormal may produce mucin. A few instances of intracranial neuroepithelial (colloid) cysts together with other lesions have been reported. The associated disorders include hydranencephaly (28); xanthogranuloma (xanthoma) in the third ventricle (35); frontoethmoidal meningoencephalocele, acqueductal stenosis, obstructive hydrocephalus, and microphthalmia (34) and congenital vascular anomalies of the brain (31). The subject of the present report is the combined occurrence of an intracranial ependymoma producing excessive mucin, and multiple neuroepithelial cysts of the choroid plexus of one lateral ventricle.

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